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leukemia

 [loo-ke´me-ah]
a progressive, malignant neoplasm of the blood-forming organs, marked by diffuse replacement of the bone marrow development of leukocytes and their precursors in the blood and bone marrow. It is accompanied by a reduced number of erythrocytes and blood platelets, resulting in anemia and increased susceptibility to infection and hemorrhage. Other typical symptoms include fever, pain in the joints and bones, and swelling of the lymph nodes, spleen, and liver. adj., adj leuke´mic.
Types of Leukemia. Leukemia is classified clinically in several ways: (1) acute versus chronic, terms that have become altered from their usual meanings and refer to the degree of cell differentiation; (2) the predominant proliferating cells: myelocytic, granulocytic, or lymphocytic; and (3) increase in or maintenance of the number of abnormal cells in the blood—preleukemic.

Acute leukemia is characterized by fatigue, headache, sore throat, and dyspnea, followed by symptoms of acute tonsillitis, stomatitis, bleeding from the mucous membranes of the mouth, alimentary canal, and rectum, and pain in the bones and joints. There eventually is enlargement of the lymph nodes, liver, and spleen. Common to all leukemias are the tendency to bleed and the resultant anemia and increased susceptibility to infection. The diagnosis of leukemia requires confirmation of leukemic cells in the bone marrow by bone marrow biopsy and aspiration. Abnormalities may also be seen in peripheral blood smears.
Treatment. The treatment of choice is systemic combination chemotherapy with a variety of antineoplastic drug regimens. The disease can also be treated by a bone marrow transplant after a remission is achieved with chemotherapy.
Patient Care. Leukemia affects almost every system within the body and can present a variety of patient care problems. Of primary concern are those symptoms attendant to suppression of normal bone marrow function, particularly susceptibility to infection due to the predominance of immature and abnormally functioning white blood cells, bleeding tendency owing to decreased platelet count, and anemia due to decreased erythrocyte count. Chronic abnormal tissue perfusion, increased need for rest, and decreased sensitivity to heat and cold require careful planning and intervention. Additionally, the patient will need relief from pain and discomfort arising from enlargement of the lymph nodes and distention of the liver and spleen.

Because of the malignant nature of leukemia and the fear and anxiety created by the knowledge that one has a form of cancer, patients and their families and significant others will need help in coping with anxiety, mental depression, and realistic fears about dying and death. The financial burden of the illness and disruption of the life of the individual and the family also impose a special burden on them. Referral to appropriate persons and agencies that can help meet their needs is an essential part of the holistic care of the patient with leukemia.
acute leukemia leukemia in which the involved cell line shows little or no differentiation, usually consisting of blast cells; two types are distinguished, acute lymphoblastic leukemia and acute myelogenous leukemia.
acute granulocytic leukemia acute myelogenous leukemia.
acute lymphoblastic leukemia (ALL) (acute lymphocytic leukemia) acute leukemia of the lymphoblastic type, one of the two major categories of acute leukemia, primarily affecting young children. Symptoms include anemia, fatigue, weight loss, easy bruising, thrombocytopenia, granulocytopenia with bacterial infections, bone pain, lymphadenopathy, hepatosplenomegaly, and sometimes spread to the central nervous system (meningism) or to other organs. There are three major subtypes: The pre–B-cell is the most common, consisting of small uniform lymphoblasts that do not synthesize complete functional immunoglobulins or synthesize heavy chains only. The B-cell type is rare and consists of lymphoblasts that express surface immunoglobulins and have a surface translocation similar to that of Burkitt's lymphoma. The T-cell type has cells that express surface antigens characteristic of T cells.
acute megakaryoblastic leukemia (acute megakaryocytic leukemia) a form of acute myelogenous leukemia in which megakaryocytes are predominant and platelets are increased in the blood, often with fibrosis; it can occur at any age. Called also megakaryoblastic or megakaryocytic leukemia.
acute monocytic leukemia an uncommon form of acute myelogenous leukemia in which the predominating cells are identified as monocytes; a few myelocytes may also be present. It can affect any age group. Called also monocytic leukemia.
acute myeloblastic leukemia
1. a common kind of acute myelogenous leukemia, in which myeloblasts predominate; it usually occurs in infants and middle-aged to older adults. Two types are distinguished; those that have minimal cell differentiation or maturation and those that have more advanced differentiation. Called also myeloblastic leukemia and acute myeloid leukemia.
acute myelocytic leukemia acute myelogenous leukemia.
acute myelogenous leukemia (AML) acute leukemia of the myelogenous type, one of the two major categories of acute leukemia; most types affect primarily middle-aged to elderly people. Symptoms include anemia, fatigue, weight loss, easy bruising, thrombocytopenia, and granulocytopenia that leads to persistent bacterial infections. Several types are distinguished, named according to the stage in which abnormal proliferation begins: acute undifferentiated l., acute myeloblastic l., acute promyelocytic l., acute myelomonocytic l., acute monocytic l., acute erythroleukemia, and acute megakaryocytic l. Called also acute myelocytic l. and acute nonlymphocytic l.
acute myeloid leukemia
acute myelomonocytic leukemia one of the more common types of acute myelogenous leukemia, characterized by both malignant monocytes and myeloblasts; it usually affects middle aged to older adults. See also chronic myelomonocytic leukemia. Called also myelomonocytic or Naegeli's leukemia.
acute nonlymphocytic leukemia acute myelogenous leukemia.
acute promyelocytic leukemia acute myelogenous leukemia in which more than half the cells are malignant promyelocytes, often associated with abnormal bleeding secondary to thrombocytopenia, hypofibrinogenemia, and decreased levels of coagulation factor V; it usually occurs in young adults. Called also promyelocytic leukemia.
acute undifferentiated leukemia acute myelogenous leukemia in which the predominating cell is so immature and primitive that it cannot be classified. Called also stem cell leukemia and undifferentiated cell leukemia.
adult T-cell leukemia (adult T-cell leukemia/lymphoma) a form of leukemia with onset in adulthood, leukemic cells with T-cell properties, frequent dermal involvement, lymphadenopathy and hepatosplenomegaly, and a subacute or chronic course; it is associated with human T-cell leukemia-lymphoma virus.
aleukemic leukemia leukemia in which the leukocyte count is normal or below normal; it may be lymphocytic, monocytic, or myelocytic.
basophilic leukemia a rare type of leukemia in which basophils predominate; both acute and chronic varieties have been observed.
blast cell leukemia acute undifferentiated leukemia.
chronic leukemia leukemia in which the involved cell line is well-differentiated, usually B-lymphocytes, but immunologically incompetent; types distinguished include chronic granulocytic, chronic lymphocytic, chronic myelomonocytic, eosinophilic, and hairy cell leukemia.
chronic granulocytic leukemia chronic leukemia of the myelogenous type, occurring mainly between the age of 25 and 60, usually associated with a unique chromosomal abnormality. The major clinical manifestations of malaise, hepatosplenomegaly, anemia, and leukocytosis are related to abnormal, excessive, unrestrained overgrowth of granulocytes in the bone marrow. Called also chronic myelocytic or chronic myeloid leukemia.
chronic lymphocytic leukemia chronic leukemia of the lymphoblastic type, a common form mainly seen in the elderly; symptoms include lymphadenopathy, fatigue, renal involvement, and pulmonary leukemic infiltrates. Circulating malignant cells are usually differentiated B-lymphocytes; a minority of cases have mixed T and B lymphocytes or entirely T-lymphocytes.
chronic myelocytic leukemia (chronic myelogenous leukemia,) (chronic myeloid leukemia) chronic granulocytic leukemia.
chronic myelomonocytic leukemia a slowly progressing form of chronic leukemia that usually affects the elderly and sometimes progresses to acute myelomonocytic leukemia. Symptoms include splenomegaly, monocytosis with granulocytosis, and thrombocytopenia.
leukemia cu´tis leukemia with leukocytic invasion of the skin marked by pink, reddish brown, or purple macules, papules, and tumors.
eosinophilic leukemia a form of leukemia in which the eosinophil is the predominating cell. Although resembling chronic granulocytic leukemia in many ways, this form may follow an acute course despite the absence of predominantly blast forms in the peripheral blood.
granulocytic leukemia myelogenous leukemia.
hairy cell leukemia a form of chronic leukemia marked by splenomegaly and by an abundance of abnormal large mononuclear cells covered by hairlike villi (hairy cells) in the bone marrow, spleen, liver, and peripheral blood. Called also leukemic reticuloendotheliosis.
leukopenic leukemia aleukemic leukemia.
lymphatic leukemia (lymphoblastic leukemia) leukemia associated with hyperplasia and overactivity of the lymphoid tissue; there are increased numbers of circulating malignant lymphocytes and lymphoblasts. See also acute lymphoblastic leukemia and chronic lymphocytic leukemia.
lymphocytic leukemia (lymphogenous leukemia) (lymphoid leukemia) lymphoblastic leukemia.
lymphosarcoma cell leukemia the B-cell type of acute lymphoblastic leukemia.
mast cell leukemia a rare type marked by overwhelming numbers of tissue mast cells in the peripheral blood.
megakaryoblastic leukemia acute megakaryocytic leukemia.
micromyeloblastic leukemia a form of myelogenous leukemia in which the immature, nucleoli-containing cells are small and are distinguishable from lymphocytes only by special staining.
monocytic leukemia acute monocytic leukemia.
myelocytic leukemia (myelogenous leukemia) (myeloid granulocytic leukemia) a form arising from myeloid tissue in which polymorphonuclear leukocytes and their precursors predominate.
myelomonocytic leukemia (Naegeli's leukemia) acute myelomonocytic leukemia.
plasma cell leukemia (plasmacytic leukemia) a rare type in which the predominating cell in the peripheral blood is the plasma cell; it is often seen in asociation with multiple myeloma.
prolymphocytic leukemia a type of chronic leukemia marked by large numbers of circulating lymphocytes, predominantly prolymphocytes, with massive splenomegaly and occasionally lymphadenopathy; prognosis is often poor.
promyelocytic leukemia acute promyelocytic leukemia.
Rieder cell leukemia a form of acute myelogenous leukemia in which the blood contains the abnormal cells called Rieder's lymphocytes, asynchronously developed lymphocytes that have immature cytoplasm and a lobulated, indented, comparatively more mature nucleus.
subleukemic leukemia aleukemic leukemia.
undifferentiated leukemia acute undifferentiated leukemia.

ALL

Abbreviation for acute lymphocytic leukemia.

ALL

acute lymphoblastic leukemia.

ALL

abbr.
1. acute lymphoblastic leukemia
2. antilymphocyte serum

ALL

1 abbreviation for acute lymphocytic leukemia.
2 abbreviation for acute lymphoid leukemia.

acute lymphoblastic leukaemia

A malignant lymphoproliferative process, which commonly affects children and young adults, affecting ± 1800/year (US); ± 650/year (UK).

Aetiology
ALL has a hereditary component; it is 20-fold increased in patients with Down syndrome; it is linked to benzene exposure, radiation therapy in ankylosing spondylitis.
 
Clinical findings
Abrupt onset, often ± 3-month history of fatigue, fever, haemorrhage from multiple sites, lymphadenopathy, hepatomegaly, splenomegaly.
 
Molecular pathology
Most are B cells and express CD19; 60% have karyotypic abnormalities; the most common cytogenetic abnormality is the cryptic t(12;21) translocation, resulting in TEL-AML fusion (25% of cases), followed by the t(1;19)(q23;p13.3) translocation, seen in 5% of cases.
 
Prognosis
90–95% achieve remission; improved cure rate is attributed to prophylaxis for meningeal leukaemia and more intense systemic chemotherapy. The current survival ranges from 20% to 75%.

FAB classification, acute leukaemias
Acute lymphocytic leukaemia (ALL)
L1—Small monotonous lymphocytes.
L2—Mixed L1- and L3-type lymphocytes.
L3—Large homogeneous blast cells.

ALL

2. Allergy.

ALL

Abbreviation for acute lymphocytic leukemia.

leukemia

, leucemia (loo-ke'me-a) [ leuko- + -emia]
Any of a class of hematological malignancies of bone marrow cells in which immortal clones of immature blood cells multiply at the expense of normal blood cells. As normal blood cells are depleted from the body, anemia, infection, hemorrhage, or death result. The leukemias are categorized as chronic or acute; by the cell type from which they originate; and by the genetic, chromosomal, or growth factor aberration present in the malignant cells.

Chronic leukemias, which have a relatively slow course, include chronic lymphocytic (CLL), chronic myelogenous or granulocytic (CML), and hairy cell leukemia (a subtype of CLL). Median survival in these illnesses is about 4 yr.

Acute leukemias include acute lymphocytic (ALL) and acute myeloid (myelogenous) (AML) leukemia. If untreated, these diseases are fatal within weeks or months. Each of these types of leukemia is discussed in subentries, below.

Etiology

All the different molecular events leading to the development of unchecked cellular reproduction in the leukemias result from genetic or chromosomal lesions in blood-forming cells. Duplications of genetic material (hyperdiploidy), loss of genetic information (hypodiploidy), inactivation of genes that normally suppress tumor development, chromosomal translocations, and the release of abnormal fusion proteins can all cause leukemia. These genetic lesions in turn can be produced by viruses, ionizing radiation, chemotherapeutic drugs, and toxic chemicals. Rarely, leukemias are caused by familial genetic syndromes (e.g., as ataxia telangiectasia, Bloom's syndrome, or Fanconi's syndrome).

Symptoms

Clinical findings such as anemia, fatigue, lethargy, fever, and bone and joint pain may be present. Physical findings include combinations of pallor, petechiae, or purpura; mucous membrane bleeding; enlarged liver, spleen, and kidneys; and tenderness over the sternum and other bones.

Diagnosis

Microscopic examination of peripheral blood and specimens of bone marrow are used to establish the diagnosis. These studies are followed by cytochemical and cytogenetic studies of abnormal cells found in the marrow or the peripheral blood to confirm the diagnosis with special stains and chromosomal analysis. Leukemic cells can also be identified by flow cytometry and immunocytochemistry, which rely on antibodies binding to and helping to identify malignant cells. The spread of leukemias to internal organs (e.g., the brain, the kidneys, or the lungs) may be evaluated with imaging tests (e.g., MRI studies, CT scans, or ultrasound).

Treatment

Chemotherapy, bone marrow transplantation, or both are used to treat leukemias. Regimens are devised regularly and are tailored to specific illnesses. Treatment is often given in several phases, with a period of induction chemotherapy to induce remission by completely eliminating leukemic cells from the bone marrow, followed by consolidation and maintenance phases. This multiphase treatment is designed to further deplete malignant cells from the bone marrow and to achieve complete cure.

Patient care

Patient care measures focus on eradicating the illness; managing complications; minimizing the effects of chemotherapy; preserving veins (often an indwelling port is inserted to administer chemotherapy); and providing comfort, education, and psychological support. The specific needs of patients (many of whom are children) and their families must be considered. Instruction is provided about drugs the patient will receive, including any adverse reactions and measures that will be taken to prevent or alleviate these effects. Prescribed chemotherapy is administered with special precautions when indicated for infusion and drug disposal. If the chemotherapy causes weight loss or anorexia, nutritional guidance is provided. Oral, skin, and rectal care must be meticulous, e.g., the nurse must thoroughly clean the skin before all invasive procedures, inspect the patient for perirectal erosions, use strict aseptic technique when starting an intravenous line, and change sets (i.e., intravenous tubing and associated equipment) according to chemotherapeutic protocols. Ports are irrigated according to agency protocol. If the patient is receiving intrathecal chemotherapy, the lumbar puncture site is checked frequently for bleeding or oozing. The patient and family are taught to recognize signs of infection (fevers, chills, sore throat, cough, urinary difficulties) and are urged to report these to the oncologist/hematologist promptly. To prevent infection in neutropenic patients, strict hand hygiene protocols, special diets, and (in hospitalized patients) laminar airflow or other reverse isolation measures are instituted. The patient is monitored for bleeding. If bleeding occurs, compresses are applied and the bleeding site is elevated. Transfusions of platelets and other blood cells are often needed. Complications associated with specific chemotherapeutic regimens (e.g., hair loss, nausea and vomiting, anemia, neutropenia, and low platelets) are explained to the patient, along with management strategies that will be employed. Prescribed analgesics are administered as needed, and noninvasive pain relief techniques and comfort measures (e.g., position changes, cutaneous stimulation, distraction, relaxation breathing, and imagery) may be used. Gentle oral hygiene measures and protective skin care are explained. Fluid intake should be increased to eliminate chemotherapy metabolites, and the patient advised to void more frequently to prevent cystitis. Dietary fiber is important, and stool softeners may be used to ensure normal bowel movements. Antidiarrheals usually control diarrhea, but the patient should be monitored for signs of dehydration. Fatigue is an anticipated adverse effect of treatment; therefore the patient is encouraged to alternate activity with rest periods and to obtain assistance with daily activities as necessary. Reproductive issues should be discussed with the patient. Patient care routines and visiting times should be flexible when hospitalization is required. The patient and family are encouraged to participate in care as much as possible. Referrals are made to social service agencies, home health care agencies, and support groups. If the patient does not respond to treatment and has reached the terminal phase of the disease, supportive nursing, palliative care, or hospice care should be discussed sensitively with patients and their caregivers.

acute lymphoblastic leukemia

Acute lymphocytic leukemia.
Enlarge picture
ACUTE LYMPHOCYTIC LEUKEMIA: Peripheral blood smear

acute lymphocytic leukemia

Abbreviation: ALL
A hematological malignancy marked by the unchecked multiplication of immature lymphoid cells in the bone marrow, blood, and body tissues. In 2008 the American Cancer Society estimated about 5400 Americans would be diagnosed with ALL. It is rapidly fatal if left untreated. Synonym: acute lymphoblastic l. See: illustration; leukemia

Etiology

Any of a wide range of acquired or congenital chromosomal abnormalities can cause ALL, including lesions that result in the release of excess growth factors from cells and those that cause the loss of cancer-suppressing genes.

Symptoms

Fatigue, lethargy, bleeding, bone and joint pain, and a predisposition to fever and infection are characteristic of ALL and other leukemias.

Diagnosis

The disease is suggested by the presence of abnormalities on the complete blood count or peripheral blood smear and is confirmed by immunophenotyping.

Treatment

In childhood, ALL induction chemotherapy often begins with steroids, vinca alkaloids, and asparaginase. This is followed, after bone marrow recovery, by consolidation chemotherapy with multidrug regimens, including high-dose methotrexate. Maintenance therapies, which may last 2 years or longer, include methotrexate, mercaptopurines, and other cytotoxic agents. Prophylaxis against central nervous system disease is accomplished by intrathecal drug administration. In referral hospitals, allogeneic stem cell transplantation is sometimes used for refractory disease. About 90% of treated children achieve remission. The 5-year survival of children with ALL is about 85%. Adult ALL is much less responsive to therapy; only about a third of adult patients are cured. In both childhood and adult ALL, allopurinol and hydration precede induction chemotherapy to prevent hyperuricemia caused by tumor lysis.

Prognosis

Late complications of therapy are not uncommon.

acute myelogenous leukemia

Abbreviation: AML
Acute myeloid leukemia.

acute myeloid leukemia

Abbreviation: AML
Any of a group of hematological malignancies in which neoplastic cells develop from myeloid, monocytic, erythrocytic, or megakaryocytic precursors. AML is four times more common in adults than acute lymphocytic leukemia (ALL). In 2008, the American Cancer Society estimated about 13,300 Americans would be diagnosed with AML, and that the disease would cause 8,800 deaths. It occasionally follows a myelodysplastic disorder or aplastic anemia and sometimes occurs as a consequence of a familial disorder of fragile chromosomes (e.g., Fanconi's syndrome).

All forms of AML are marked by neoplastic replacement of normal bone marrow and circulation of immature cells (“blasts”) in the peripheral blood. Anemia and thrombocytopenia commonly occur. The central nervous system and other organs are occasionally invaded. Complete remissions occur in approximately 65% of treated patients; responses to treatment lasting 5 years are achieved in 15% to 25% of treated patients. Synonym: acute myelogenous l.; acute nonlymphocytic l.

Etiology

Genetic and chromosomal aberrations, such as are found in other leukemias, are characteristic.

Symptoms

Exertional fatigue as a result of anemia, bleeding due to thrombocytopenia, and infections due to a lack of normal white blood cells are common.

Treatment

Cytotoxic chemotherapies, with an induction phase followed by consolidation, are used. Typically, cytosine arabinoside and an anthracycline are used during induction for AML. Allogeneic bone marrow transplantation is used when a matching donor is available; stem cell transplantation is an option for some patients with specific cytogenetic abnormalities.

acute nonlymphocytic leukemia

Abbreviation: ANLL
Acute myeloid leukemia.

aleukemic leukemia

Leukemia cutis.
Enlarge picture
CHRONIC LYMPHOCYTIC LEUKEMIA: Peripheral blood smear

chronic lymphocytic leukemia

Abbreviation: CLL
A malignancy in which abnormal lymphocytes (usually B cells) proliferate and infiltrate body tissues, often causing lymph node enlargement and immune dysfunction. Infectious complications are common. Median life expectancy is about 4 years. Chronic lymphocytic leukemia is the most common leukemia in industrialized nations. It usually occurs in people (older men) above age 60. Its incidence rises to 20 cases per 100,000 in people over 80. In 2008 the American Cancer Society estimated that 15,100 people would be diagnosed with CLL and that 4,400 would die of the disease. The timing of treatment and the prognosis in CLL depend on the stage of the disease. Staging includes such factors as the number of abnormal lymphocytes in the bloodstream, how quickly they double, and the presence of lymphadenopathy, organomegaly, or cytopenias. See: illustration

Treatment

Patients with advanced stages of the illness are often treated with chlorambucil, fludarabine, or other cytotoxic agents, often with rituximab (a monoclonal antibody) added to enhance response. Patients with early-stage disease are not usually given therapy.

chronic myelogenous leukemia

Abbreviation: CML
Chronic myeloid leukemia.

chronic myeloid leukemia

Abbreviation: CML
A hematological malignancy marked by a sustained increase in the number of granulocytes, splenic enlargement, and a specific cytogenetic anomaly (the “Philadelphia chromosome”) in the bone marrow of more than 90% of patients. The disease affects one or two people per 100,000. In 2008 the American Cancer Society estimated that 4830 people would be diagnosed with CML and that 450 would die of the disease. The course of the disease has three phases: a chronic one in which blood counts are relatively easy to control with medications; an accelerated phase in which granulocyte counts become more resistant to chemotherapy; and a “blast” crisis, which resembles acute leukemia. Median survival is about 4 years. It generally occurs between ages 40 and 50, affecting slightly more men than women (4600 adults in the U.S. in 2005). Synonym: chronic myelogenous l See: leukemia

Etiology

CML results from a translocation of genetic material between chromosomes 9 and 22. The translocation results in the production of an abnormal tyrosine kinase that makes affected cells immortal.

Symptoms

CML often is diagnosed in asymptomatic patients who are found to have an unexplained leukocytosis when their complete blood counts are checked. Subsequent evaluation, including bone marrow aspiration and biopsy with cytogenetic analysis, reveal the Philadelphia chromosome.

Treatment

Imatinib mesylate (a drug that blocks an abnormal kinase made by Philadelphia chromosome positive CML cells) effectively reduces the number of tumor cells in the chronic phase of CML to normal in nearly 90% of patients. An alternative is stem cell transplantation.

leukemia cutis

An invasion of the dermis and subcutaneous fat by leukemic cells. The invasion often happens before these cells proliferate in the bone marrow or are detectable in the peripheral blood. The cells may cause several different types of skin rashes, including blue nodules (giving the skin a “blueberry muffin” appearance), papules, plaques, and ulcers.
Synonym: aleukemic l
Enlarge picture
HAIRY CELL LEUKEMIA: Bone marrow aspirate
Enlarge picture
LYMPHOCYTES IN HAIRY CELL LEUKEMIA

hairy cell leukemia

Abbreviation: HCL
A chronic, low-grade hematological malignancy of abnormally shaped B lymphocytes (“hairy cells”). The disease is marked by pancytopenia and splenomegaly. Median survival in untreated patients is about 5 years. The disease is rare, being only 1% to 2% of all leukemias. The median age of patients is 50 years; men are affected more commonly than women by a 4-to-1 ratio. See: illustration

Symptoms

Weight loss, hypermetabolism, infectious complications, and abdominal discomfort due to splenic enlargement are common.

Treatment

Cladribine, pentostatin, interferon alfa, and rituximab (a monoclonal antibody) are representative chemotherapeutic options.

illustration

mixed-lineage leukemia

Abbreviation: MLL
An aggressive, primarily childhood leukemia caused by the translocation of a gene from chromosome 11 to a region that overproduces fusion proteins.

Enlarge picture
ACUTE LYMPHOCYTIC LEUKEMIA: Peripheral blood smear

acute lymphocytic leukemia

Abbreviation: ALL
A hematological malignancy marked by the unchecked multiplication of immature lymphoid cells in the bone marrow, blood, and body tissues. In 2008 the American Cancer Society estimated about 5400 Americans would be diagnosed with ALL. It is rapidly fatal if left untreated. Synonym: acute lymphoblastic l. See: illustration; leukemia

Etiology

Any of a wide range of acquired or congenital chromosomal abnormalities can cause ALL, including lesions that result in the release of excess growth factors from cells and those that cause the loss of cancer-suppressing genes.

Symptoms

Fatigue, lethargy, bleeding, bone and joint pain, and a predisposition to fever and infection are characteristic of ALL and other leukemias.

Diagnosis

The disease is suggested by the presence of abnormalities on the complete blood count or peripheral blood smear and is confirmed by immunophenotyping.

Treatment

In childhood, ALL induction chemotherapy often begins with steroids, vinca alkaloids, and asparaginase. This is followed, after bone marrow recovery, by consolidation chemotherapy with multidrug regimens, including high-dose methotrexate. Maintenance therapies, which may last 2 years or longer, include methotrexate, mercaptopurines, and other cytotoxic agents. Prophylaxis against central nervous system disease is accomplished by intrathecal drug administration. In referral hospitals, allogeneic stem cell transplantation is sometimes used for refractory disease. About 90% of treated children achieve remission. The 5-year survival of children with ALL is about 85%. Adult ALL is much less responsive to therapy; only about a third of adult patients are cured. In both childhood and adult ALL, allopurinol and hydration precede induction chemotherapy to prevent hyperuricemia caused by tumor lysis.

Prognosis

Late complications of therapy are not uncommon.

See also: leukemia

ALL,

n.pr See leukemia, acute lymphocytic.
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