Hodgkin's Disease Hodgkin's disease is a rare lymphoma, a cancer of the lymphatic system.
disease /dis·ease/ (dĭ-zēz´) any deviation from or interruption of the normal structure or function of any body part, organ, or system that is manifested by a characteristic set of symptoms and signs and whose etiology, pathology, and prognosis may be known or unknown. See also entries under syndrome.
Hodgkin's disease, Hodgkin's lymphoma
Hodgkin's disease [hoj´kinz]
Hodgkin's disease, or Hodgkin's lymphoma, was first described in 1832 by Thomas Hodgkin, a British physician. Hodgkin clearly differentiated between this disease and the much more common non-Hodgkin's lymphomas. Prior to 1970, few individuals survived Hodgkin's disease. Now, however, the majority of individuals with this cancer can be cured.
The lymphatic system is part of the body's immune system, for fighting disease, and a part of the blood-producing system. It includes the lymph vessels and nodes, and the spleen, bone marrow, and thymus. The narrow lymphatic vessels carry lymphatic fluid from throughout the body. The lymph nodes are small organs that filter the lymphatic fluid and trap foreign substances, including viruses, bacteria, and cancer cells. The spleen, in the upper left abdomen, removes old cells and debris from the blood. The bone marrow, the tissue inside the bones, produces new red and white blood cells.
Lymphocytes are white blood cells that recognize and destroy disease-causing organisms. Lymphocytes are produced in the lymph nodes, spleen, and bone marrow. They circulate throughout the body in the blood and lymphatic fluid. Clusters of immune cells also exist in major organs.
Hodgkin's disease is a type of lymphoma in which antibody-producing cells of the lymphatic system begin to grow abnormally. It usually begins in a lymph node and progresses slowly, in a fairly predictable way, spreading via the lymphatic vessels from one group of lymph nodes to the next. Sometimes it invades organs that are adjacent to the lymph nodes. If the cancer cells spread to the blood, the disease can reach almost any site in the body. Advanced cases of Hodgkin's disease may involve the spleen, liver, bone marrow, and lungs.
There are different subtypes of Hodgkin's disease:
- nodular sclerosis (30-60% of cases)
- mixed cellularity (20-40% of cases)
- lymphocyte predominant (5-10% of cases)
- lymphocyte depleted (less than 5% of cases)
- unclassified
The American Cancer Society estimates that there will be 7,400 new cases of Hodgkin's disease in the United States in 2001—3,500 in females and 3,900 in males. It is estimated that 700 men and 600 women in the United States will die of the disease in 2001.
Hodgkin's disease can occur at any age. However, the majority of cases develop in early adulthood (ages 15-40) and late adulthood (after age 55). Approximately 10-15% of cases are in children under age 17. It is more common in boys than in girls under the age of 10. The disease is very rare in children under five.
The cause of Hodgkin's disease is not known. It is suspected that some interaction between an individual's genetic makeup, environmental exposures, and infectious agents may be responsible. Immune system deficiencies also may be involved.
Early symptoms of Hodgkin's disease may be similar to those of the flu:
- fevers, night sweats, chills
- fatigue
- loss of appetite
- weight loss
- itching
- pain after drinking alcoholic beverages
- swelling of one or more lymph nodes
Sudden or emergency symptoms of Hodgkin's disease include:
- sudden high fever
- loss of bladder and/or bowel control
- numbness in the arms and legs and a loss of strength
As lymph nodes swell, they may push on other structures, causing a variety of symptoms:
- pain due to pressure on nerve roots
- loss of function in muscle groups served by compressed nerves
- coughing or shortness of breath due to compression of the windpipe and/or airways, by swollen lymph nodes in the chest
- kidney failure from compression of the ureters, the tubes that carry urine from the kidneys to the bladder
- swelling in the face, neck, or legs, due to pressure on veins
- paralysis in the legs due to pressure on the spinal cord
As Hodgkin's disease progresses, the immune system becomes less effective at fighting infection. Thus, patients with Hodgkin's lymphoma become more susceptible to both common infections caused by bacteria and unusual (opportunistic) infections. Later symptoms of Hodgkin's disease include the formation of tumors.
Significantly, as many as 75% of individuals with Hodgkin's disease do not have any typical symptoms.
As with many forms of cancer, diagnosis of Hodgkin's disease has two major components.
- identification of Hodgkin's lymphoma as the cause of the patient's disease
- staging of the disease to determine how far the cancer has spread
The initial diagnosis of Hodgkin's disease often results from abnormalities in a chest x ray that was performed because of nonspecific symptoms. The physician then takes a medical history to check for the presence of symptoms and conducts a complete physical examination.
The size, tenderness, firmness, and location of swollen lymph nodes are determined and correlated with any signs of infection. In particular, lymph nodes that do not shrink after treatment with antibiotics may be a cause for concern. The lymph nodes that are most often affected by Hodgkin's disease include those of the neck, above the collarbone, under the arms, and in the chest above the diaphragm.
Diagnosis of Hodgkin's disease requires either the removal of an entire enlarged lymph node (an excisional biopsy) or an incisional biopsy, in which only a small part of a large tumor is removed. If the node is near the skin, the biopsy is performed with a local anesthetic. However, if it is inside the chest or abdomen, general anesthesia is required.
The sample of biopsied tissue is examined under a microscope. Giant cells called Reed-Sternberg cells must be present to confirm a diagnosis of Hodgkin's disease. These cells, which usually contain two or more nuclei, are named for the two pathologists who discovered them. Normal cells have only one nucleus (the organelle within the cell that contains the genetic material). Affected lymph nodes may contain only a few Reed-Sternberg cells and they may be difficult to recognize. Characteristics of other types of cells in the biopsied tissue help to diagnose the subtype of Hodgkin's disease.
A fine needle aspiration (FNA) biopsy, in which a thin needle and syringe are used to remove a small amount of fluid and bits of tissue from a tumor, has the advantage of not requiring surgery. An FNA may be performed prior to an excisional or incisional biopsy, to check for infection or for the spread of cancer from another organ. However an FNA biopsy does not provide enough tissue to diagnose Hodgkin's disease.
Occasionally, additional biopsies are required to diagnose Hodgkin's disease. In rare instances, other tests, that detect certain substances on the surfaces of cancer cells or changes in the DNA of cells, are used to distinguish Hodgkin's disease from non-Hodgkin's lymphoma.
Staging is very important in Hodgkin's disease. This is because the cancer usually spreads in a predictable pattern, without skipping sets of lymph nodes until late in the progression of the disease.
Imaging of the abdomen, chest, and pelvis is used to identify areas of enlarged lymph nodes and abnormalities in the spleen or other organs. Computerized axial tomography (CT or CAT) scans use a rotating x-ray beam to obtain pictures. Magnetic resonance imaging (MRI) uses magnetic fields and radio waves to produce images of the body. Chest x rays also may be taken. These images will reveal rounded lumps called nodules in the affected lymph nodes and other organs.
Another imaging technique for Hodgkin's disease is a gallium scan, in which the radioactive element gallium is injected into a vein. The cancer cells take up the gallium and a special camera that detects the gallium is used to determine the location and size of tumors. Gallium scans are used when Hodgkin's disease is in the chest and may be hard to detect by other methods. Gallium scans also are used to monitor progress during treatment.
A lymphangiogram, a radiograph of the lymphatic vessels, involves injecting a dye into a lymphatic vessel in the foot. Tracking of the dye locates the disease in the abdomen and pelvis. This method is used less frequently and is usually not used with children.
Positron emission tomography (PET) scans are an extremely accurate method for staging Hodgkin's disease. A very low dose of radioactive glucose, a sugar, is injected into the body. The glucose travels to metabolically active sites, including cancerous regions that require large amounts of glucose. The PET scan detects the radioactivity and produces images of the entire body that distinguish between cancerous and non-cancerous tissues.
Anemia (a low red-blood-cell count), fevers, or night sweats are indications that Hodgkin's disease may be in the bone marrow. In these cases, a bone-marrow biopsy, in which a large needle is used to remove a narrow, cylindrical piece of bone, may be necessary to determine the spread of the cancer. Alternatively, an aspiration, in which a needle is used to remove small bits of bone marrow, may be used. The marrow usually is removed from the back of the hip or other large bone.
Sometimes further staging, called pathological staging or a staging laparotomy, is used for Hodgkin's disease. In this operation, a surgeon checks the abdominal lymph nodes and other organs for cancer and removes small pieces of tissue. A pathologist examines the tissue samples for Hodgkin's disease cells. Usually the spleen is removed (a splenectomy) during the laparotomy. The splenectomy helps with staging Hodgkin's disease, as well as removing a disease site.
All of the available treatments for Hodgkin's disease have serious side effects, both short and long-term. However, with accurate staging, physicians and patients often can choose the minimum treatment that will cure the disease. The staging system for Hodgkin's disease is the Ann Arbor Staging Classification, also called the Cotswold System or the Revised Ann Arbor System.
Hodgkin's disease is divided into four stages, with additional substages:
- Stage I: The disease is confined to one lymph node area
- Stage IE: The disease extends from the one lymph node area to adjacent regions
- Stage II: The disease is in two or more lymph node areas on one side of the diaphragm (the muscle below the lungs)
- Stage IIE: The disease extends to adjacent regions of at least one of these nodes
- Stage III: The disease is in lymph node areas on both sides of the diaphragm
- Stage IIIE/IIISE: The disease extends into adjacent areas or organs (IIIE) and/or the spleen (IIISE)
- Stage IV: The disease has spread from the lymphatic system to one or more other organs, such as the bone marrow or liver
Treatment for Hodgkin's disease depends both on the stage of the disease and whether or not symptoms are present. Stages are labeled with an A if no symptoms are present. If symptoms are present, the stage is labeled with a B. These symptoms include:
- loss of more than 10% of body weight over the previous six months
- fevers above 100 (37.70 C) degrees F
- drenching night sweats
Radiation therapy and/or chemotherapy (drug therapy) are the standard treatments for Hodgkin's disease. If the disease is confined to one area of the body, radiotherapy is usually used. This treatment, with x rays or other high-energy rays, also is used when the disease is in bulky areas such as the chest, where chemotherapeutic drugs cannot reach all of the cancer. External-beam radiation, a focused beam from an external machine, is used to irradiate only the affected lymph nodes. This procedure is called involved field radiation.
More advanced stages of Hodgkin's disease may be treated with mantle field radiation, in which the lymph nodes of the neck, chest, and underarms are irradiated. Inverted Y field radiation is used to irradiate the spleen and the lymph nodes in the upper abdomen and pelvis. Total nodal irradiation includes both mantle field and inverted Y field radiation.
Since external-beam radiation damages healthy tissue near the cancer cells, the temporary side effects of radiotherapy can include sunburn-like skin damage, fatigue, nausea, and diarrhea. Other temporary side effects may include a sore throat and difficulty swallowing. Long-term side effects depend on the dose and the location of the radiation and the age of the patient. Since radiation of the ovaries causes permanent sterility (the inability to have offspring), the ovaries of girls and young women are protected during radiotherapy. Sometimes the ovaries are surgically moved from the region to be irradiated.
If the Hodgkin's disease has progressed to additional lymph nodes or other organs, or if there is a recurrence of the disease within two years of radiation treatment, chemotherapy is used.
Chemotherapy utilizes a combination of drugs, each of which kills cancer cells in a different way. The most common chemotherapy regimens for Hodgkin's disease are MOPP (either mechlorethamine or methotrexate with Oncovin, procarbazine, prednisone) and ABVD (Adriamycin or doxorubicin, bleomycin, vincristine, dacarbazine). Each of these consists of four different drugs. ABVD is used more frequently than MOPP because it has fewer severe side effects. However MOPP is used for individuals who are at risk for heart failure. The chemotherapeutic drugs may be injected into a vein or muscle, or taken orally, as a pill or liquid.
Children who are sexually mature when they develop Hodgkin's disease, and whose muscle and bone mass are almost completely developed, usually receive the same treatment as adults. Younger children usually are treated with chemotherapy, since radiation will adversely affect bone and muscle growth. However, radiation may be used in low dosages, in combination with chemotherapy. The chemotherapy for children with Hodgkin's disease usually includes more drugs than ABVD and MOPP.
The side effects of chemotherapy for Hodgkin's disease depend on the dose of drugs and the length of time they are taken. Since these drugs target rapidly dividing cancer cells, they also affect normal cells that grow rapidly. These include the cells of the bone marrow, the linings of the mouth and intestines, and hair follicles. Damage to bone marrow leads to lower white blood cell counts and lower resistance to infection. It also leads to lower red blood cell counts, which can result in fatigue and easy bleeding and bruising. Damage to intestinal cells leads to a loss of appetite, nausea, and vomiting. Mouth sores and hair loss also are common side effects of chemotherapy. These side effects disappear when the chemotherapy is discontinued. Some drugs can reduce or prevent the nausea and vomiting.
Chemotherapy for Hodgkin's disease may lead to long-term complications. The drugs may damage the heart, lungs, kidneys, and liver. In children, growth may be impeded. Some chemotherapy can cause sterility, so men may choose to have their sperm frozen prior to treatment. Women may stop ovulating and menstruating during chemotherapy. This may or may not be permanent.
Treatment for higher-stage Hodgkin's disease often involves a combination of radiotherapy and chemotherapy. Following three or four chemotherapy regimens, involved field radiation may be directed at the most affected areas of the body. The long-term side effects often are more severe when radiation and chemotherapy are used in combination.
The development of a second type of cancer is the most serious risk from radiation and chemotherapy treatment for Hodgkin's disease. In particular, there is a risk of developing leukemia, breast cancer, bone cancer, or thyroid cancer. Chemotherapy, particularly MOPP, or chemotherapy in conjunction with radiotherapy, significantly increases the risk for leukemia.
Following treatment, the original diagnostic tests for Hodgkin's disease are repeated, to determine whether all traces of the cancer have been eliminated and to check for long-term side effects of treatment. In resistant Hodgkin's disease, some cancer cells remain following treatment. If the cancer continues to spread during treatment, it is called progressive Hodgkin's disease. If the disease returns after treatment, it is known as recurrent Hodgkin's disease. It may recur in the area where it first started or elsewhere in the body. It may recur immediately after treatment or many years later.
Additional treatment is necessary with these types of Hodgkin's disease. If the initial treatment was radiation therapy alone, chemotherapy may be used, or vice versa. Chemotherapy with different drugs, or higher doses, may be used to treat recurrent Hodgkin's. However, radiation to the same area is never repeated.
An autologous bone marrow and/or a peripheral blood stem cell transplantation (PBSCT) often is recommended for treating resistant or recurrent Hodgkin's disease, particularly if the disease recurs within a few months of a chemotherapy-induced remission. These transplants are autologous because they utilize the individual's own cells. The patient's bone marrow cells or peripheral blood stem cells (immature bone marrow cells found in the blood) are collected and frozen prior to high-dosage chemotherapy, which destroys bone marrow cells. A procedure called leukapheresis is used to collect the stem cells. Following the high-dosage chemotherapy, and possibly radiation, the bone marrow cells or stem cells are reinjected into the individual.
Most complementary therapies for Hodgkin's disease are designed to stimulate the immune system to destroy cancer cells and repair normal cells that have been damaged by treatment. These therapies are used in conjunction with standard treatment.
Immunologic therapies, also known as immunotherapies, biological therapies, or biological response modifier therapies, utilize substances that are produced by the immune system. These include interferon (an immune system protein), monoclonal antibodies (specially engineered antibodies), colony-stimulating (growth) factors (such as filgrastim), and vaccines. Many immunotherapies for Hodgkin's disease are experimental and available only through clinical trials. These biological agents may have side effects.
Coenzyme Q10 (CoQ10) and polysaccharide K (PSK) are being evaluated for their ability to stimulate the immune system and protect healthy tissue, as well as possible anti-cancer activities. Camphor, also known as 714-X, green tea, and hoxsey (which is a mixture of a number of substances), have been promoted as immune system enhancers. However there is no evidence that they are effective against Hodgkin's disease. Hoxsey, in particular, can produce serious side effects.
Hodgkin's disease, particularly in children, is one of the most curable forms of cancer. Approximately 90% of individuals are cured of the disease with chemotherapy and/or radiation.
The one-year relative survival rate following treatment for Hodgkin's disease is 93%. Relative survival rates do not include individuals who die of causes other than Hodgkin's disease. The percentage of individuals who have not died of Hodgkin's disease within five years of diagnosis is 90-95% for those with stage I or stage II disease. The figure is 85-90% for those diagnosed with stage III Hodgkin's and approximately 80% for those diagnosed with stage IV disease. The 15-year relative survival rate is 63%. Approximately 75% of children are alive and cancer free 20 years after the original diagnosis of Hodgkin's.
Acute myelocytic leukemia, a very serious cancer, may develop in as many as 2-6% of individuals receiving certain types of treatment for Hodgkin's disease. Women under the age of 30 who are treated with radiation to the chest have a much higher risk for developing breast cancer. Both men and women are at higher risk for developing lung or thyroid cancers as a result of chest irradiation.
Individuals with the type of Hodgkin's disease known as nodular lymphocytic predominance have a 2% chance of developing non-Hodgkin's lymphoma. Apparently, this is a result of the Hodgkin's disease itself and not the treatment.
disease /dis·ease/ (dĭ-zēz´) any deviation from or interruption of the normal structure or function of any body part, organ, or system that is manifested by a characteristic set of symptoms and signs and whose etiology, pathology, and prognosis may be known or unknown. See also entries under syndrome.
acquired cystic disease of kidney the development of cysts in the formerly noncystic failing kidney in end-stage renal disease.
Addison's disease bronzelike pigmentation of the skin, severe prostration, progressive anemia, low blood pressure, diarrhea, and digestive disturbance, due to adrenal hypofunction.
Albers-Schönberg disease osteopetrosis.
allogeneic disease graft-versus-host reaction occurring in immunosuppressed animals receiving injections of allogeneic lymphocytes.
Alpers' disease a rare disease of young children, characterized by neuronal deterioration of the cerebral cortex and elsewhere, progressive mental deterioration, motor disturbances, seizures, and early death.
alpha chain disease heavy chain disease characterized by plasma cell infiltration of the lamina propria of the small intestine resulting in malabsorption with diarrhea, abdominal pain, and weight loss, possibly accompanied by pulmonary involvement.
Alzheimer's disease progressive degenerative disease of the brain, of unknown cause; characterized by diffuse atrophy throughout the cerebral cortex with distinctive histopathological changes.
Andersen's disease glycogen storage d., type IV.
apatite deposition disease a connective tissue disorder marked by deposition of hydroxyapatite crystals in one or more joints or bursae.
Aran-Duchenne disease spinal muscular atrophy.
arteriosclerotic cardiovascular disease (ASCVD) atherosclerotic involvement of arteries to the heart and to other organs, resulting in debility or death; sometimes used specifically for ischemic heart disease.
arteriosclerotic heart disease (ASHD) ischemic heart d.
autoimmune disease any of a group of disorders in which tissue injury is associated with humoral or cell-mediated responses to the body's own constituents; they may be systemic or organ-specific.
Ayerza's disease polycythemia vera with chronic cyanosis, dyspnea, bronchitis, bronchiectasis, hepatosplenomegaly, bone marrow hyperplasia, and pulmonary artery sclerosis.
Banti's disease congestive splenomegaly.
Barlow disease scurvy in infants.
Barraquer's disease partial lipodystrophy.
Basedow's disease Graves' d.
Batten disease , Batten-Mayou disease
2. more generally, any or all of the group of disorders constituting neuronal ceroid lipofuscinosis.
Bayle's disease general paresis.
Bazin's disease erythema induratum.
Bekhterev's (Bechterew's) disease ankylosing spondylitis.
Benson's disease asteroid hyalosis.
Berger's disease IgA glomerulonephritis.
Bernhardt's disease , Bernhardt-Roth disease meralgia paresthetica.
Besnier-Boeck disease sarcoidosis.
Best's disease congenital macular degeneration.
Bielschowsky-Janský disease Janský-Bielschowsky d.
Binswanger's disease a degenerative dementia of presenile onset caused by demyelination of the subcortical white matter of the brain.
black disease a fatal disease of sheep, and sometimes of humans, in the United States and Australia, due to Clostridium novyi, marked by necrotic areas in the liver.
Blocq's disease astasia-abasia.
Blount disease tibia vara.
Boeck's disease sarcoidosis.
Bornholm disease epidemic pleurodynia.
Bowen's disease a squamous cell carcinoma in situ, often due to prolonged exposure to arsenic; usually occurring on sun-exposed areas of skin. The corresponding lesion on the glans penis is termed erythroplasia of Queyrat.
Brill's disease Brill-Zinsser d.
Brill-Symmers disease giant follicular lymphoma.
Brill-Zinsser disease mild recrudescence of epidemic typhus years after the initial infection, because Rickettsia prowazekii has persisted in body tissue in an inactive state, with humans as the reservoir.
broad beta disease familial dysbetalipoproteinemia; named for the electrophoretic mobility of the abnormal chylomicron and very-low-density lipoprotein remnants produced.
Busse-Buschke disease cryptococcosis.
Caffey's disease infantile cortical hyperostosis.
calcium hydroxyapatite deposition disease apatite deposition d.
calcium pyrophosphate deposition disease (CPDD) an acute or chronic inflammatory arthropathy caused by deposition of calcium pyrophosphate dihydrate (CPPD) crystals in the joints, chondrocalcinosis, and crystals in the synovial fluid. Acute attacks are sometimes called pseudogout.
Calvé-Perthes disease osteochondrosis of capitular epiphysis of femur.
Camurati-Engelmann disease diaphyseal dysplasia.
Canavan disease , Canavan-van Bogaert-Bertrand disease spongy degeneration of the central nervous system.
Carrión's disease bartonellosis.
Castleman disease a benign or premalignant condition resembling lymphoma but without recognizable malignant cells; there are isolated masses of lymphoid tissue and lymph node hyperplasia, usually in the abdominal or mediastinal area.
cat-scratch disease a usually benign, self-limited disease of the regional lymph nodes, caused by Bartonella henselae and characterized by a papule or pustule at the site of a cat scratch, subacute painful regional lymphadenitis, and mild fever.
celiac disease a malabsorption syndrome precipitated by ingestion of gluten-containing foods, with loss of villous structure of the proximal intestinal mucosa, bulky, frothy diarrhea, abdominal distention, flatulence, weight loss, and vitamin and electrolyte depletion.
Chagas disease trypanosomiasis due to Trypanosoma cruzi; its course may be acute, subacute, or chronic.
Charcot-Marie-Tooth disease muscular atrophy of variable inheritance, beginning in the muscles supplied by the peroneal nerves and progressing to those of the hands and arms.
cholesteryl ester storage disease (CESD) a lysosomal storage disease due to deficiency of lysosomal cholesterol esterase, variably characterized by some combination of hepatomegaly, hyperbetalipoproteinemia, and premature atherosclerosis.
Christmas disease hemophilia B.
chronic granulomatous disease frequent, severe infections of the skin, oral and intestinal mucosa, reticuloendothelial system, bones, lungs, and genitourinary tract associated with a genetically determined defect in the intracellular bactericidal function of leukocytes.
chronic obstructive pulmonary disease (COPD) any disorder marked by persistent obstruction of bronchial air flow.
Coats' disease exudative retinopathy.
collagen disease any of a group of diseases characterized by widespread pathologic changes in connective tissue; they include lupus erythematosus, dermatomyositis, scleroderma, polyarteritis nodosa, thrombotic purpura, rheumatic fever, and rheumatoid arthritis. Cf. collagen disorder.
communicable disease a disease the causative agents of which may pass or be carried from one person to another directly or indirectly.
Concato's disease progressive malignant polyserositis with large effusions into the pericardium, pleura, and peritoneum.
constitutional disease one involving a system of organs or one with widespread symptoms.
Cori's disease glycogen storage d., type III.
coronary artery disease (CAD) atherosclerosis of the coronary arteries, which may cause angina pectoris, myocardial infarction, and sudden death; risk factors include hypercholesterolemia, hypertension, smoking, diabetes mellitus, and low levels of high-density lipoproteins.
coronary heart disease (CHD) ischemic heart d.
Cowden disease a hereditary disease marked by multiple ectodermal, mesodermal, and endodermal nevoid and neoplastic anomalies.
Creutzfeldt-Jakob disease a rare prion disease existing in sporadic, familial, and infectious forms, with onset usually in middle life, and having a wide variety of clinical and pathological features. The most commonly seen are spongiform degeneration of neurons, neuronal loss, gliosis, and amyloid plaque formation, accompanied by rapidly progressive dementia, myoclonus, motor disturbances, and encephalographic changes, with death occurring usually within a year of onset.
Crigler-Najjar disease see under syndrome.
Crohn's disease regional enteritis; a chronic granulomatous inflammatory disease usually in the terminal ileum with scarring and thickening of the wall, often leading to intestinal obstruction and formation of fistulas and abscesses.
Crouzon's disease craniofacial dysostosis.
Cruveilhier's disease spinal muscular atrophy.
Cushing's disease Cushing's syndrome in which the hyperadrenocorticism is secondary to excessive pituitary secretion of adrenocorticotropic hormone.
cystic disease of breast mammary dysplasia with formation of blue dome cysts.
cytomegalic inclusion disease , cytomegalovirus disease an infection due to cytomegalovirus and marked by nuclear inclusion bodies in enlarged infected cells. In the congenital form, there is hepatosplenomegaly with cirrhosis, and microcephaly with mental or motor retardation. Acquired disease may cause a clinical state similar to infectious mononucleosis. When acquired by blood transfusion, postperfusion syndrome results.
deficiency disease a condition caused by dietary or metabolic deficiency, including all diseases due to an insufficient supply of essential nutrients.
degenerative joint disease osteoarthritis.
Dejerine's disease , Dejerine-Sottas disease progressive hypertrophic neuropathy.
demyelinating disease any condition characterized by destruction of the myelin sheaths of nerves.
disappearing bone disease gradual resorption of a bone or group of bones, sometimes associated with multiple hemangiomas, usually in children or young adults and following trauma.
diverticular disease a general term including the prediverticular state, diverticulosis, and diverticulitis.
Duchenne's disease
3. tabes dorsalis.
Duchenne-Aran disease spinal muscular atrophy.
Duhring's disease dermatitis herpetiformis.
Dukes' disease a febrile disease of childhood marked by an exanthematous eruption, probably due to a virus of the Coxsackie-ECHO group.
Durand-Nicolas-Favre disease lymphogranuloma venereum.
Duroziez's disease congenital mitral stenosis.
Ebola virus disease fatal acute hemorrhagic fever resembling Marburg virus disease but caused by Ebola virus, seen in the Sudan and Zaire.
Ebstein's disease see under anomaly.
end-stage renal disease chronic irreversible renal failure.
Erb's disease Duchenne's muscular dystrophy.
Erb-Goldflam disease myasthenia gravis.
Eulenburg's disease paramyotonia congenita.
extrapyramidal disease any of a group of clinical disorders marked by abnormal involuntary movements, alterations in muscle tone, and postural disturbances; they include parkinsonism, chorea, athetosis, etc.
Fabry's disease an X-linked lysosomal storage disease of glycosphingolipid catabolism resulting from deficiency of α-galactosidase A and leading to accumulation of ceramide trihexoside in the cardiovascular and renal systems.
Farber's disease a lysosomal storage disease due to defective ceramidase and characterized by hoarseness, aphonia, dermatitis, bone and joint deformities, granulomatous reaction, and psychomotor retardation.
Fazio-Londe disease a rare type of progressive bulbar palsy occurring in childhood.
Feer disease acrodynia.
fibrocystic disease of breast a form of mammary dysplasia with formation of cysts of various size containing a semitransparent, turbid fluid that imparts a brown to blue color to the unopened cysts; believed due to abnormal hyperplasia of the ductal epithelium and dilatation of the ducts of the mammary gland, resulting from exaggeration and distortion of normal menstrual cycle–related breast changes.
fibrocystic disease of the pancreas cystic fibrosis.
fifth disease erythema infectiosum.
flint disease chalicosis.
floating beta disease familial dysbetalipoproteinemia.
focal disease a localized disease.
foot-and-mouth disease an acute, contagious viral disease of wild and domestic cloven-footed animals and occasionally humans, marked by vesicular eruption on the lips, buccal cavity, pharynx, legs, and feet.
Forbes' disease glycogen storage d., type III.
fourth disease Dukes' d.
fourth venereal disease granuloma inguinale.
Fox-Fordyce disease a persistent and recalcitrant, itchy, papular eruption, chiefly of the axillae and pubes, due to inflammation of apocrine sweat glands.
Freiberg's disease osteochondrosis of the head of the second metatarsal bone.
Friedländer's disease endarteritis obliterans.
Friedreich's disease paramyoclonus multiplex.
functional disease see under disorder.
Garré's disease sclerosing nonsuppurative osteomyelitis.
gastroesophageal reflux disease (GERD) any condition resulting from gastroesophageal reflux, characterized by heartburn and regurgitation; see also reflux esophagitis.
Gaucher's disease a hereditary disorder of glucocerebroside metabolism, marked by the presence of Gaucher's cells in the marrow, and by hepatosplenomegaly and erosion of the cortices of long bones and pelvis. The adult form is associated with moderate anemia and thrombocytopenia, and yellowish pigmentation of the skin; in the infantile form there is, in addition, marked central nervous system impairment; in the juvenile form there are rapidly progressive systemic manifestations but moderate central nervous system involvement.
genetic disease a general term for any disorder caused by a genetic mechanism, comprising chromosome aberrations (or anomalies), mendelian (or monogenic or single-gene) disorders, and multifactorial disorders.
gestational trophoblastic disease see under neoplasia.
Gilbert disease a familial, benign elevation of bilirubin levels without evidence of liver damage or hematologic abnormalities.
Gilles de la Tourette's disease see under syndrome.
Glanzmann disease see thrombasthenia.
glycogen storage disease any of a number of rare inborn errors of metabolism caused by defects in specific enzymes or transporters involved in the metabolism of glycogen.
type I glucose-6-phosphatase deficiency: a severe hepatorenal form due to deficiency of the hepatic enzyme glucose-6-phosphatase, resulting in liver and kidney involvement, with hepatomegaly, hypoglycemia, hyperuricemia, and gout
type IA glycogen storage d., type I.
type IB a form resembling type I but additionally predisposing to infection due to neutropenia and to chronic inflammatory bowel disease; due to a defect in the transport system for glucose 6-phosphate.
type II a disorder due to deficiency of the lysosomal enzyme α-1,4,-glucosidase, the severe infant form resulting in generalized glycogen accumulation, with cardiomegaly, cardiorespiratory failure, and death, and a milder adult form being a gradual skeletal myopathy that sometimes causes respiratory problems.
type III a form due to deficiency of debrancher enzyme (amylo-1,6-glucosidase) in muscle, liver, or both; defects in the liver enzyme are characterized by hepatomegaly and hypoglycemia while defects in the muscle enzyme are characterized by progressive muscle wasting and weakness.
type IV brancher enzyme deficiency; cirrhosis of the liver, hepatosplenomegaly, progressive hepatic failure, and death due to deficiency of the glycogen brancher enzyme (1,4-α-glucan branching enzyme).
type V muscle cramps and fatigue during exercise due to a defect in the skeletal muscle isozyme of glycogen phosphorylase (muscle phosphorylase).
type VI hepatomegaly, mild to moderate hypoglycemia and mild ketosis, due to deficiency of the liver isozyme of glycogen phosphorylase (hepatic phosphorylase).
type VII muscle weakness and cramping after exercise due to deficiency of the muscle isozyme of 6-phosphofructokinase.
type VIII phosphorylase .
graft-versus-host (GVH) disease disease caused by the immune response of histoincompatible, immunocompetent donor cells against the tissue of immunocompromised host, as a complication of bone marrow transplantation, or as a result of maternal-fetal blood transfusion, or therapeutic transfusion to an immunocompromised recipient.
Graves' disease an association of hyperthyroidism, goiter, and exophthalmos, with accelerated pulse rate, profuse sweating, nervous symptoms, psychic disturbances, emaciation, and elevated basal metabolism.
Greenfield's disease former name for the late infantile form of metachromatic leukodystrophy.
Gull's disease atrophy of the thyroid gland with myxedema.
Günther disease congenital erythropoietic porphyria.
H disease Hartnup d.
Hailey-Hailey disease benign familial pemphigus.
Hallervorden-Spatz disease an autosomal recessive disorder caused by decreased numbers of myelin sheaths of the globus pallidus and substantia nigra, with accumulation of iron pigment, progressive rigidity beginning in the legs, choreoathetoid movements, dysarthria, and mental deterioration.
Hand's disease Hand-Schüller-Christian d.
hand-foot-and-mouth disease a mild, highly infectious viral disease of children, with vesicular lesions in the mouth and on the hands and feet.
Hand-Schüller-Christian disease a chronic, progressive form of multifocal Langerhans cell histiocytosis, sometimes with accumulation of cholesterol, characterized by the triad of calvarial bone defects, exophthalmos, and diabetes insipidus.
Hansen's disease leprosy.
Hartnup disease a hereditary disorder of intestinal and renal transport of neutral α-amino acids, marked by a pellagra-like skin rash, with transient cerebellar ataxia, constant renal aminoaciduria, and other biochemical abnormalities.
Hashimoto's disease a progressive disease of the thyroid gland with degeneration of its epithelial elements and replacement by lymphoid and fibrous tissue.
heavy chain diseases a group of malignant neoplasms of lymphoplasmacytic cells marked by the presence of immunoglobulin heavy chains or heavy chain fragments; they are classified according to heavy chain type, e.g., alpha chain disease.
Heine-Medin disease the major form of poliomyelitis.
hemoglobin disease any of various hereditary molecular diseases characterized by abnormal hemoglobins in the red blood cells; the homozygous form is manifested by hemolytic anemia.
hemolytic disease of the newborn erythroblastosis fetalis.
hemorrhagic disease of the newborn a self-limited hemorrhagic disorder of the first few days of life, due to deficiency of vitamin K–dependent coagulation factors II, VII, IX, and X.
Hers' disease glycogen storage d., type VI.
Heubner-Herter disease the infantile form of celiac disease.
hip-joint disease tuberculosis of the hip joint.
Hippel's disease von Hippel's d.
Hirschsprung's disease congenital megacolon.
His disease , His-Werner disease trench fever.
Hodgkin's disease a form of malignant lymphoma marked clinically by painless, progressive enlargement of lymph nodes, spleen, and general lymphoid tissue; other symptoms may include anorexia, lassitude, weight loss, fever, pruritus, night sweats, and anemia. Reed-Sternberg cells are characteristically present. Four types have been distinguished on the basis of histopathologic criteria.
hoof-and-mouth disease foot-and-mouth d.
hookworm disease infection with the hookworm Ancylostoma duodenale or Necator americanus, whose larvae enter the body through the skin or in contaminated food or water and migrate to the small intestine where, as adults, they attach to the mucosa and ingest blood; symptoms may include abdominal pain, diarrhea, colic or nausea, and anemia.
hyaline membrane disease a type of respiratory distress syndrome of the newborn in which there is formation of a hyaline-like membrane lining the terminal respiratory passages; extensive atelectasis is attributed to lack of surfactant.
hydatid disease an infection, usually of the liver, due to larval forms of tapeworms of the genus Echinococcus, marked by development of expanding cysts.
hypophosphatemic bone disease an inherited disorder resembling a mild form of X-linked hypophosphatemia, similarly due to a defect in renal tubular function but usually showing osteomalacia without radiographic evidence of rickets.
immune complex disease local or systemic disease caused by the formation of circulating immune complexes and their deposition in tissue, due to activation of complement and to recruitment and activation of leukocytes in type III hypersensitivity reactions.
infectious disease one due to organisms ranging in size from viruses to parasitic worms; it may be contagious in origin, result from nosocomial organisms, or be due to endogenous microflora from the nose and throat, skin, or bowel.
inflammatory bowel disease any idiopathic inflammatory disease of the bowel, such as Crohn's disease and ulcerative colitis.
intercurrent disease one occurring during the course of another disease with which it has no connection.
iron storage disease hemochromatosis.
ischemic bowel disease ischemic colitis.
ischemic heart disease (IHD) any of a group of acute or chronic cardiac disabilities resulting from insufficient supply of oxygenated blood to the heart.
Janský-Bielschowsky disease the late infantile form of neuronal ceroid lipofuscinosis, occurring between two and four years of age, characterized by abnormal accumulation of lipofuscin; beginning as myoclonic seizures and progressing to neurologic and retinal deterioration and death by age 8 to 12.
jumping disease any of several culture-specific disorders characterized by exaggerated responses to small stimuli, muscle tics including jumping, obedience even to dangerous suggestions, and sometimes coprolalia or echolalia.
juvenile Paget disease hyperostosis corticalis deformans juvenilis.
Kashin-Bek (Kaschin-Beck) disease a disabling degenerative disease of the peripheral joints and spine, endemic in northeastern Asia; believed to be caused by ingestion of cereal grains infected with the fungus Fusarium sporotrichiella.
Katayama disease schistosomiasis japonica.
Kawasaki disease a febrile illness usually affecting infants and young children, with conjunctival injection, changes to the oropharyngeal mucosa, changes to the peripheral extremities including edema, erythema, and desquamation, a primarily truncal polymorphous exanthem, and cervical lymphadenopathy. It is often associated with vasculitis of the large coronary vessels.
Kienböck's disease slowly progressive osteochondrosis of the lunate bone; it may affect other wrist bones.
kinky hair disease Menkes' syndrome.
Köhler's bone disease
1. osteochondrosis of the tarsal navicular bone in children.
2. thickening of the shaft of the second metatarsal bone and changes about its articular head, with pain in the second metatarsophalangeal joint on walking or standing.
Krabbe's disease a lysosomal storage disease beginning in infancy, due to deficiency of β-galactosidase. Pathologically, there is rapidly progressive cerebral demyelination and large globoid bodies (swollen with accumulated cerebroside) in the white substance.
Kufs' disease the adult form of neuronal ceroid lipofuscinosis, with onset prior to age 40; characterized by progressive neurologic deterioration but not blindness, excessive storage of lipofuscin, and shortened life expectancy;
Kümmell's disease compression fracture of vertebra, with symptoms a few weeks after injury, including spinal pain, intercostal neuralgia, lower limb motor disturbances, and kyphosis.
Kyasanur Forest disease a fatal viral disease of monkeys in the Kyasanur Forest of India, communicable to humans, in whom it produces hemorrhagic symptoms.
Kyrle's disease a chronic disorder of keratinization marked by keratotic plugs that develop in hair follicles and eccrine ducts, penetrating the epidermis and extending down into the corium, causing foreign-body reaction and pain.
Lafora's disease see under epilepsy.
Leber's disease
legionnaires' disease an often fatal bacterial infection caused by Legionella pneumophila, not spread by person-to-person contact, characterized by high fever, gastrointestinal pain, headache, and pneumonia; there may also be involvement of the kidneys, liver, and nervous system.
Leiner's disease a disorder of infancy characterized by generalized seborrhea-like dermatitis and erythroderma, severe intractable diarrhea, recurrent infections, and failure to thrive.
Leriche disease post-traumatic osteoporosis.
Letterer-Siwe disease a Langerhans cell histiocytosis of early childhood, of autosomal recessive inheritance, characterized by cutaneous lesions resembling seborrheic dermatitis, hemorrhagic tendency, hepatosplenomegaly, lymphadenitis, and progressive anemia. If untreated it is rapidly fatal. Called also acute disseminated Langerhans cell histiocytosis.
Libman-Sacks disease see under endocarditis.
Lindau's disease , Lindau-von Hippel disease von Hippel-Lindau d.
Little's disease congenital spastic stiffness of the limbs, a form of cerebral palsy due to lack of development of the pyramidal tracts.
Lobstein's disease see osteogenesis imperfecta.
Lou Gehrig disease amyotrophic lateral sclerosis.
Lowe disease oculocerebrorenal syndrome.
Lutz-Splendore-Almeida disease paracoccidioidomycosis.
Lyme disease a recurrent multisystemic disorder caused by the spirochete Borrelia burgdorferi, the vectors being the ticks Ixodes scapularis and I. pacificus; usually initially characterized by lesions of erythema chronicum migrans, followed by various manifestations including arthritis of the large joints, myalgia, and neurologic and cardiac abnormalities.
lysosomal storage disease an inborn error of metabolism with (1) a defect in a specific lysosomal enzyme; (2) intracellular accumulation of an unmetabolized substrate; (3) clinical progression affecting multiple tissues or organs; (4) considerable phenotypic variation within a disease.
MAC disease complex d.
McArdle disease glycogen storage d., type V.
mad cow disease bovine spongiform encephalopathy.
maple bark disease hypersensitivity pneumonitis in logging and sawmill workers due to inhalation of spores of a mold, Cryptostroma corticale, growing under the maple bark.
maple syrup urine disease (MSUD) a hereditary enzyme defect in metabolism of branched chain amino acids, marked clinically by mental and physical retardation, severe ketoacidosis, feeding difficulties, and a characteristic maple syrup odor in the urine and on the body.
Marburg virus disease a severe, often fatal, viral hemorrhagic fever first reported in Marburg, Germany, among laboratory workers exposed to African green monkeys.
Marchiafava-Micheli disease paroxysmal nocturnal hemoglobinuria.
Marie-Bamberger disease hypertrophic pulmonary osteoarthropathy.
Marie-Strümpell disease ankylosing spondylitis.
Marie-Tooth disease Charcot-Marie-Tooth d.
Mediterranean disease thalassemia major.
medullary cystic disease familial juvenile nephronophthisis.
Meniere's disease deafness, tinnitus, and dizziness, in association with nonsuppurative disease of the labyrinth.
mental disease see under disorder.
Merzbacher-Pelizaeus disease Pelizaeus-Merzbacher d.
metabolic disease one caused by a disruption of a normal metabolic pathway because of a genetically determined enzyme defect.
Meyer's disease adenoid vegetations of the pharynx.
Mikulicz's disease benign, self-limited lymphocytic infiltration and enlargement of the lacrimal and salivary glands of uncertain etiology.
Milroy disease hereditary permanent lymphedema of the legs due to lymphatic obstruction.
Minamata disease a severe neurologic disorder due to alkyl mercury poisoning, with permanent neurologic and mental disabilities or death; once prevalent among those eating contaminated seafood from Minamata Bay, Japan.
minimal change disease subtle alterations in kidney function demonstrable by clinical albuminuria and the presence of lipid droplets in cells of the proximal tubules, seen primarily in young children.
mixed connective tissue disease a combination of scleroderma, myositis, systemic lupus erythematosus, and rheumatoid arthritis, and marked serologically by the presence of antibody against extractable nuclear antigen.
Möbius disease ophthalmoplegic migraine.
molecular disease any disease in which the pathogenesis can be traced to a single molecule, usually a protein, which is either abnormal in structure or present in reduced amounts.
Mondor's disease phlebitis affecting the large subcutaneous veins normally crossing the lateral chest wall and breast from the epigastric or hypochondriac region to the axilla.
Monge's disease chronic mountain sickness.
Morquio's disease , Morquio-Ullrich disease see under syndrome.
motor neuron disease , motor system disease any disease of a motor neuron, including spinal muscular atrophy, progressive bulbar paralysis, amyotrophic lateral sclerosis, and lateral sclerosis.
Mycobacterium avium complex disease MAC disease; systemic disease caused by infection with organisms of the Mycobacterium avium-intracellulare complex in patients with human immunodeficiency virus infection.
Newcastle disease a viral disease of birds, including domestic fowl, transmissible to humans, characterized by respiratory, gastrointestinal or pulmonary, and encephalitic symptoms.
new variant Creutzfeldt-Jakob disease (nvCJD) a variant of Creutzfeldt-Jakob disease having a younger age of onset than is seen in Creutzfeldt-Jakob disease, and caused by the same agent that causes bovine spongiform encephalopathy.
Nicolas-Favre disease lymphogranuloma venereum.
Niemann's disease , Niemann-Pick disease a lysosomal storage disease due to sphingomyelin accumulation in the reticuloendothelial system; there are five types distinguished by age of onset, amount of central nervous system involvement, and degree of enzyme deficiency.
nil disease minimal change d.
Norrie's disease an X-linked disorder consisting of bilateral blindness from retinal malformation, mental retardation, and deafness.
notifiable disease one required to be reported to federal, state, or local health officials when diagnosed, because of infectiousness, severity, or frequency of occurrence.
oasthouse urine disease methionine malabsorption syndrome.
obstructive small airways disease chronic bronchitis with irreversible narrowing of the bronchioles and small bronchi with hypoxia and often hypercapnia.
occupational disease disease due to various factors involved in one's employment.
Oguchi's disease a form of hereditary night blindness and fundus discoloration following light adaptation.
organic disease one associated with demonstrable change in a bodily organ or tissue.
Osgood-Schlatter disease osteochondrosis of the tuberosity of the tibia.
Osler's disease
Owren's disease parahemophilia.
Paget's disease
1. (of bone) osteitis deformans.
2. (of breast) an intraductal inflammatory carcinoma of the breast, involving the areola and nipple.
3. an extramammary counterpart of Paget's disease (2), usually involving the vulva, and sometimes other sites, as the perianal and axillary regions.
Parkinson's disease a slowly progressive form of parkinsonism, usually seen late in life, marked by masklike facies, tremor of resting muscles, slowing of voluntary movements, festinating gait, peculiar posture, muscular weakness, and sometimes excessive sweating and feelings of heat.
Parrot's disease see under pseudoparalysis.
parrot disease psittacosis.
Parry's disease Graves' d.
Pelizaeus-Merzbacher disease a progressive familial form of leukoencephalopathy, marked by nystagmus, ataxia, tremor, parkinsonian facies, dysarthria, and mental deterioration.
Pellegrini's disease , Pellegrini-Stieda disease calcification of the medial collateral ligament of the knee due to trauma.
pelvic inflammatory disease (PID) any pelvic infection involving the upper female genital tract beyond the cervix.
periodontal disease any disease or disorder of the periodontium.
Perthes' disease osteochondrosis of capitular femoral epiphysis.
Peyronie's disease induration of the corpora cavernosa of the penis, producing a painful fibrous chordee and penile curvature.
Pfeiffer's disease infectious mononucleosis.
Pick's disease
1. progressive atrophy of the cerebral convolutions in a limited area (lobe) of the brain, with clinical manifestations and course similar to Alzheimer's disease.
polycystic kidney disease , polycystic disease of kidneys either of two unrelated heritable disorders marked by cysts in both kidneys: the autosomal dominant or adult form is more common, appears in adult life, and is marked by loss of renal function that can be either rapid or slow; the autosomal recessive or infantile form is more rare, may be congenital or may appear later in childhood, and almost always progresses to renal failure.
polycystic renal disease polycystic kidney d..
Pompe's disease glycogen storage d., type II.
Pott's disease spinal tuberculosis.
primary electrical disease serious ventricular tachycardia, and sometimes ventricular fibrillation, in the absence of recognizable structural heart disease.
prion disease any of a group of fatal, transmissible neurodegenerative diseases, which may be sporadic, familial, or acquired, caused by abnormalities of prion protein metabolism resulting from mutations in the prion protein gene or from infection with pathogenic forms of the protein.
pulseless disease Takayasu's arteritis.
Raynaud's disease a primary or idiopathic vascular disorder, most often affecting women, marked by bilateral attacks of Raynaud's phenomenon.
Recklinghausen's disease
2. (of bone) osteitis fibrosa cystica generalisata.
Refsum's disease an inherited disorder of lipid metabolism, characterized by accumulation of phytanic acid, chronic polyneuritis, retinitis pigmentosa, cerebellar ataxia, and persistent elevation of protein in cerebrospinal fluid.
remnant removal disease familial dysbetalipoproteinemia.
reversible obstructive airway disease a condition characterized by bronchospasm reversible by intervention, as in asthma.
rheumatic heart disease the most important manifestation and sequel to rheumatic fever, consisting chiefly of valvular deformities.
rheumatoid disease a systemic condition best known by its articular involvement (rheumatoid arthritis) but emphasizing nonarticular changes, e.g., pulmonary interstitial fibrosis, pleural effusion, and lung nodules.
Ritter's disease dermatitis exfoliativa neonatorum.
Roger's disease a ventricular septal defect; the term is usually restricted to small, asymptomatic defects.
runt disease a graft-versus-host disease produced by immunologically competent cells in a foreign host that is unable to reject them, resulting in gross retardation of host development and in death.
Salla disease an inherited disorder of sialic acid metabolism characterized by accumulation of sialic acid in lysosomes and excretion in the urine, mental retardation, delayed motor development, and ataxia.
Sandhoff's disease a type of GM2 gangliosidosis resembling Tay-Sachs disease, seen in non-Jews, marked by a progressively more rapid course, and due to a defect in hexosaminidase, both isozymes A and B.
Schamberg's disease a slowly progressive purpuric and pigmentary disease of the skin affecting chiefly the shins, ankles, and dorsa of the feet.
Schilder's disease subacute or chronic leukoencephalopathy in children and adolescents, similar to adrenoleukodystrophy; massive destruction of the white substance of the cerebral hemispheres leads to blindness, deafness, bilateral spasticity, and mental deterioration.
Schönlein's disease see under purpura.
secondary disease
1. one subsequent to or as a consequence of another disease.
2. one due to introduction of incompatible, immunologically competent cells into a host rendered incapable of rejecting them by heavy exposure to ionizing radiation.
self-limited disease one that runs a limited and definite course.
serum disease see under sickness.
severe combined immunodeficiency disease (SCID) see under immunodeficiency .
sexually transmitted disease venereal disease; any of a diverse group of infections transmitted by sexual contact; in some this is the only important mode of transmission, and in others transmission by nonsexual means is possible.
sickle cell disease any disease associated with the presence of hemoglobin S.
Simmonds' disease see panhypopituitarism.
sixth disease exanthema subitum.
small airways disease chronic obstructive bronchitis with irreversible narrowing of the bronchioles and small bronchi. See also obstructive small airways d.
Smith-Strang disease methionine malabsorption syndrome.
Spielmeyer-Vogt disease Vogt-Spielmeyer d.
Steinert's disease myotonic dystrophy.
Still's disease juvenile rheumatoid arthritis.
storage disease a metabolic disorder in which a specific substance (a lipid, a protein, etc.) accumulates in certain cells in unusually large amounts.
storage pool disease a blood coagulation disorder due to failure of the platelets to release adenosine diphosphate (ADP) in response to aggregating agents; characterized by mild bleeding episodes, prolonged bleeding time, and reduced aggregation response to collagen or thrombin.
Strümpell's disease
1. hereditary lateral sclerosis with the spasticity mainly limited to the legs.
Strümpell-Leichtenstern disease hemorrhagic encephalitis.
Strümpell-Marie disease ankylosing spondylitis.
Sutton's disease
1. halo nevus.
Swift's disease , Swift-Feer disease acrodynia.
Takayasu's disease see under arteritis .
Tangier disease a familial disorder characterized by a deficiency of high-density lipoproteins in the blood serum, with storage of cholesteryl esters in tissues.
Tarui's disease glycogen storage d., type VII.
Tay-Sachs disease (TSD) the most common GM2 gangliosidosis, seen almost exclusively in northeastern European Jews, characterized by infantile onset, doll-like facies, cherry-red macular spot, early blindness, hyperacusis, macrocephaly, seizures, hypotonia, and death in early childhood.
Thomsen's disease myotonia congenita.
thyrotoxic heart disease heart disease associated with hyperthyroidism, marked by atrial fibrillation, cardiac enlargement, and congestive heart failure.
transmissible neurodegenerative disease prion d..
trophoblastic disease gestational trophoblastic neoplasia.
tsutsugamushi disease scrub typhus.
tunnel disease decompression sickness.
uremic bone disease renal osteodystrophy.
venereal disease sexually transmitted d.
venoocclusive disease of the liver symptomatic occlusion of the small hepatic venules caused by ingestion of Senecio tea or related substances, by certain chemotherapy agents, or by radiation.
vinyl chloride disease acro-osteolysis resulting from exposure to vinyl chloride, characterized by Raynaud's phenomenon and skin and bony changes on the limbs.
Vogt-Spielmeyer disease the juvenile form of neuronal ceroid lipofuscinosis with onset between ages 5 and 10 years; characterized by rapid cerebroretinal degeneration, excessive neuronal storage of lipofuscin, and death within 10 to15 years.
Volkmann's disease congenital deformity of the foot due to tibiotarsal dislocation.
von Hippel's disease hemangiomatosis confined principally to the retina; when associated with hemangioblastoma of the cerebellum, it is known as von Hippel-Lindau d.
von Hippel-Lindau disease a hereditary condition marked by hemangiomas of the retina and hemangioblastomas of the cerebellum, sometimes with similar lesions of the spinal cord and cysts of the viscera; there may be neurologic symptoms such as seizures and mental retardation.
von Willebrand's disease an autosomal dominant bleeding disorder characterized by prolonged bleeding time, deficiency of von Willebrand's factor, and often impairment of adhesion of platelets on glass beads, associated with epistaxis and increased bleeding after trauma or surgery, menorrhagia, and postpartum bleeding.
Waldenström's disease osteochondrosis of the capitular femoral epiphysis.
Weber-Christian disease nodular nonsuppurative panniculitis.
Werlhof's disease idiopathic thrombocytopenic purpura.
Wernicke's disease see under encephalopathy.
Westphal-Strümpell disease hepatolenticular degeneration.
Whipple's disease a malabsorption syndrome marked by diarrhea, steatorrhea, skin pigmentation, arthralgia and arthritis, lymphadenopathy, central nervous system lesions, and infiltration of the intestinal mucosa with macrophages containing PAS-positive material.
Whitmore's disease melioidosis.
Wilson's disease an inherited, progressive disorder of copper metabolism, with accumulation of copper in liver, brain, kidney, cornea, and other tissues; it is characterized by cirrhosis in the liver, degenerative changes in the brain, and a pigmented ring at the outer margin of the cornea.
Wolman's disease a lysosomal storage disease due to deficiency of the lysosomal sterol esterase, occurring in infants, and associated with hepatosplenomegaly, adrenal steatorrhea, calcification, abdominal distention, anemia, and inanition.
woolsorter's disease inhalational anthrax.
Hodg·kin's disease (h j k nz)n. A malignant, progressive, sometimes fatal disease of unknown etiology, marked by enlargement of the lymph nodes, spleen, and liver and often accompanied by anemia and fever. |
Hodgkin's disease, Hodgkin's lymphoma
[hoj′kinz]
Etymology: Thomas Hodgkin, English physician, 1798-1866
a malignant disorder characterized by painless, progressive enlargement of lymphoid tissue, usually first evident in cervical lymph nodes; splenomegaly; and the presence of Sternberg-Reed cells, large, atypical macrophages with multiple or hyperlobulated nuclei and prominent nucleoli. Symptoms include anorexia, weight loss, generalized pruritus, low-grade fever, night sweats, anemia, and leukocytosis. The disease is diagnosed in about 7100 Americans annually and causes approximately 1700 deaths a year, affects twice as many males as females, and most often occurs in individuals 25-30 years of age and older than 50 years of age. The diagnosis is established by biopsy. The patient undergoes staging to determine the extent of the disease, including computed tomography of the chest and abdomen, complete blood count, biopsy of distant lymph nodes, liver function studies, and bilateral bone marrow biopsies. Radiotherapy, using a covering mantle to protect other organs, is the treatment of choice for early stages of the disease; combination chemotherapy is the treatment for advanced disease. Long-term remissions are achieved in more than half of the patients treated, and 60% to 90% of those with localized disease may be cured. There is a threefold increased risk of development of Hodgkin's disease in first-degree relatives, suggesting an unknown genetic mechanism.
Hodgkin's disease [hoj´kinz]
a form of malignant lymphoma characterized by painless, progressive enlargement of the lymph nodes, spleen, and lymphoid tissues generally. It often begins in a cervical lymph node on the side of the neck and spreads in a contiguous fashion through the body. It accounts for less than 1 per cent of all malignancies in the United States and is seen more in developed countries than in developing countries and more in males than in females. An unusual characteristic is that it has two peaks on its incidence curve, one between 15 and 35 years of age and the other between 55 and 75. chemotherapy and radiation therapy have increased the survival rate. The rate of survival depends on the stage at which treatment is begun; in Stages I and II the five-year survival rate is 85 per cent. Called also Hodgkin's lymphoma
Signs and Symptoms. The first sign of the disease usually is an enlargement of lymph nodes in the cervical, axillary, or inguinal chains. Severe itching is often an early symptom of the disorder; signs may also include fever, night sweats, and weight loss of greater than 10 per cent.
As Hodgkin's disease progresses, it spreads through the lymphatic system, involving other lymph nodes elsewhere in the body as well as the spleen, liver, and bone marrow. The lymph nodes and the spleen and liver may swell, and by obstructing other organs may cause coughing, breathlessness, or enlargement of the abdomen. The patient often becomes neutropenic, thrombocytopenic, or anemic and because of blood changes the body becomes less able to combat infections.
There are several different staging systems, classifying the disease according to stages of development of malignancy; the stages are helpful in establishing the prognosis and prescribing treatment. The most commonly used is the Cotswold or Modified Ann Arbor Staging System. In Stage I only one localized lymph node region is involved. Stage IE indicates involvement of a single extralymphatic organ. Stage II indicates two or more involved nodes on the same side of the diaphragm. Stage IIE indicates involvement of an extralymphatic organ and one or more nodes on the same side of the diaphragm. IIS designates splenic involvement with localization below the diaphragm. In Stage III there is disease on both sides of the diaphragm, sometimes with splenic involvement (IIIS) or extralymphatic organ involvement (IIIE), or both (IIISE). In Stage IV there is diffuse involvement of one or more extralymphatic organs or tissues with or without associated lymph node involvement. The presence or absence of systemic manifestations should be indicated by adding the letter A or B to the stage number, where A indicates asymptomatic and B indicates fever, night sweats, and weight loss of more than 10 per cent of body weight.
As Hodgkin's disease progresses, it spreads through the lymphatic system, involving other lymph nodes elsewhere in the body as well as the spleen, liver, and bone marrow. The lymph nodes and the spleen and liver may swell, and by obstructing other organs may cause coughing, breathlessness, or enlargement of the abdomen. The patient often becomes neutropenic, thrombocytopenic, or anemic and because of blood changes the body becomes less able to combat infections.
There are several different staging systems, classifying the disease according to stages of development of malignancy; the stages are helpful in establishing the prognosis and prescribing treatment. The most commonly used is the Cotswold or Modified Ann Arbor Staging System. In Stage I only one localized lymph node region is involved. Stage IE indicates involvement of a single extralymphatic organ. Stage II indicates two or more involved nodes on the same side of the diaphragm. Stage IIE indicates involvement of an extralymphatic organ and one or more nodes on the same side of the diaphragm. IIS designates splenic involvement with localization below the diaphragm. In Stage III there is disease on both sides of the diaphragm, sometimes with splenic involvement (IIIS) or extralymphatic organ involvement (IIIE), or both (IIISE). In Stage IV there is diffuse involvement of one or more extralymphatic organs or tissues with or without associated lymph node involvement. The presence or absence of systemic manifestations should be indicated by adding the letter A or B to the stage number, where A indicates asymptomatic and B indicates fever, night sweats, and weight loss of more than 10 per cent of body weight.
Diagnosis. The diagnosis of Hodgkin's disease requires the histologic identification of the characteristic malignant cell of the disease, the Reed-Sternberg cell. The accepted histopathologic classification distinguishes four different disease patterns: lymphocyte predominance, mixed cellularity, lymphocyte depletion, and nodular sclerosis. The evaluation required for staging includes a history and physical examination; CT scans of the chest, abdomen, and pelvis; laboratory tests, including a complete blood count, serum alkaline phosphatase, and liver and kidney function tests; and sometimes gallium scanning or lymphangiography; and a surgical lymph node biopsy. Osseous involvement is evaluated with bone marrow biopsy and bone scans. The evaluation of abdominal involvement may require exploratory laparotomy or splenotomy.
Treatment and Care. If the disease is localized (Stages IA, IIA, or IIIA), the treatment of choice is radiation therapy capable of delivering a high dose of radiation deep into the tissues. chemotherapy is recommended for patients with systemic involvement (Stages IB, IIB, and IIIB). The chemotherapeutic agents are administered in combination and intermittently so that there is a synergistic cytotoxic effect without overlapping toxicities. Drugs are chosen according to their effect on different phases of cell growth and proliferation. (See also antineoplastic therapy.) Often it is decided to employ both chemotherapy and radiation therapy, especially in treating bulky tumor involvement of nodes and spleen.
After recovery, follow-up care is extremely important. There is an increased risk of developing other cancers later in life.
After recovery, follow-up care is extremely important. There is an increased risk of developing other cancers later in life.
Staging of Hodgkin's disease. From Damjanov, 1996.
Hodgkin's disease
a primary lymph node neoplastic disease of humans. Rarely in animals, mainly dogs, do lymphoid neoplasms satisfy the criteria, e.g. Reed-Sternberg cells, for diagnosis as Hodgkin's lymphoma.
Hodgkin's disease
Hodgkin's lymphoma Hematology A type of lymphoma most common in young adults, accounting for ± 0.7% of all new cancers in the US–±7500 cases; HD is clinically distinct as it often responds
well to chemotherapy, and histologically distinct as its sine qua non diagnosis requires Reed-Sternberg cells Clinical Asymptomatic enlargement of lymph nodes, spleen, or other lymphoid tissue, ±fever, weight loss, fatigue, night
sweats Staging Ann Arbor classification Treatment RT, MOPP, mechlorethamine, vincristine, procarbazine, prednisone; 20% are treatment 'failures' and may respond to various cocktails–eg, ABVD–doxorubicin, bleomycin,
vinblastine, dacarbazine, salvage therapy, third line therapy. See Ann Arbor classification, Reed-Sternberg cell.
Hodgkin's disease stages
I CA present in one lymphoid region or in only one extranodal region or organ
II CA present in two or more lymphoid regions on same side of diaphragm, or cancer is found in only one extranodal region or organ and in surrounding lymph nodes; other lymph node areas on the same side of the diaphragm may also
be involved
III CA present in lymphoid tissues on both sides of diaphragm, and may have spread to an area or organ near lymphoid regions and/or to spleen
IV CA has spread to extranodal organ/organs, on both sides of diaphragm
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