Hodgkin lymphoma


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Hodg·kin dis·ease

(hoj'kin),
a disease marked by chronic enlargement of the lymph nodes, often local at the onset and later generalized, together with enlargement of the spleen and often of the liver, no pronounced leukocytosis, and commonly anemia and continuous or remittent (Pel-Ebstein) fever; considered to be a malignant neoplasm of lymphoid cells of uncertain origin (Reed-Sternberg cells), associated with inflammatory infiltration of lymphocytes and eosinophilic leukocytes and fibrosis; can be classified into lymphocytic predominant, nodular sclerosing, mixed cellularity, and lymphocytic depletion types; a similar disease occurs in domestic cats.
Synonym(s): Hodgkin lymphoma, lymphadenoma (2)

Hodgkin lymphoma

(hŏj′kĭn) or

Hodgkin's lymphoma

(-kĭnz)
n.
A lymphoma typically characterized by the presence of Reed-Sternberg cells and producing symptoms that include enlarged lymph nodes and spleen, fever, night sweats, weight loss, and itching. Also called Hodgkin's disease.

Hodgkin, Thomas

(hoj'kin)
Brit. physician, 1798–1866.

Hodgkin disease

Abbreviation: HD
A malignant lymphoma whose pathological hallmark is the Reed-Sternberg (RS) cell. The disease may affect persons of any age but occurs most often in adults in their early 30s. Its incidence is higher in males than in females. It is slightly more common in Caucasians than in other racial groups. The disease has a bimodal age distribution: it is common in people between the ages of 15 and 35 and in another group of patients older than 50. About 7500 new cases of the disease are diagnosed annually in the U.S. This lymphoma typically begins in a single lymph node (esp. in the neck, axilla, groin, or near the aorta) and spreads to adjacent nodes if it is not recognized and treated early. It may metastasize gradually to lymphatic tissue on both sides of the diaphragm or disseminate widely to tissues outside the lymph nodes. The degree of metastasis defines the stage of the disease; early disease (stage I or II) is present in one or a few lymph nodes, whereas widespread disease has disseminated to both sides of the diaphragm (stage III) or throughout the body (stage IV). The lower the stage of the disease, the better the prognosis. Patients with stage I Hodgkin lymphoma have a 90% survival rate 5 years after diagnosis. Synonym: Hodgkin lymphomanon-Hodgkin lymphoma; Reed-Sternberg cell;

Etiology

Epstein-Barr virus has been found in the cells of nearly half of all patients with Hodgkin disease.

Symptoms

Early stage patients may have no symptoms other than a painless lump or enlarged gland in the armpit or neck. Others may develop fevers, night sweats, loss of appetite, and weight loss.

Diagnosis

The presence of the giant, multinucleated RS cell in tissue obtained for biopsy is diagnostic.

Treatment

The goal of therapy is cure, not only palliation of symptoms. Treatment depends on accurate staging. Combinations of radiation therapy with chemotherapy have been traditionally used (radiation alone for stages I and II, radiation and chemotherapy for stage III, and chemotherapy for stage IV), although chemotherapies that rely on multiple agents may be as effective. Autologous bone marrow transplant or autologous peripheral blood stem cell transfusion (along with high-dose chemotherapy) also has been used in treatment, esp. among younger patients. Antiemetics, sedatives, antidiarrheals, and antipyretic drugs are given for patient comfort.

Patient care

All procedures and treatments associated with the plan of care are explained. The patient is assessed for nutritional deficiencies and malnutrition by obtaining regular weight readings, checking anthropomorphic measurements, and monitoring appropriate laboratory studies (e.g., serum protein levels, transferrin levels) and, as necessary, using anergy panels. A well-balanced, high-calorie, high-protein diet is provided. The patient is observed for complications during chemotherapy, including anorexia, nausea, vomiting, mouth ulcers, alopecia, fatigue, and bone marrow depression as well as for adverse reactions to radiation therapy, such as hair loss, anorexia, nausea, vomiting, and fatigue. Supportive care is given as indicated for adverse reactions to chemotherapy or radiation therapy. Comfort measures are provided to promote relaxation, and periods of rest are planned because the patient tires easily. Hematological studies are followed closely during treatment, and colony-stimulating factors are administered as necessary to stimulate red and white blood cell production. Antiemetic drugs are administered as prescribed. The importance of gentle but thorough oral hygiene to prevent stomatitis is stressed. To control pain and bleeding, a soft toothbrush or sponge-stick (toothette), cotton swabs, and a soothing or anesthetic mouthwash, such as a sodium bicarbonate mixture or viscous lidocaine, are used as prescribed. The patient can apply petroleum jelly to the lips and should avoid astringent mouthwashes. He or she is advised to pace activities to counteract therapy-induced fatigue and is taught relaxation techniques to promote comfort and rest and reduce anxiety. The patient should avoid crowds and any person with a known infection and notify the health care provider if any signs or symptoms of infection develop. Health care providers should stay with the patient during periods of stress and anxiety and provide emotional support to the patient and family. Referral to local support groups may be helpful. Women of childbearing age should delay pregnancy until long-term remission occurs. Follow-up care includes regular examinations with an oncologist and blood tests or radiographic studies to assess for disease recurrence. As necessary, both patient and family are referred for respite or hospice care. The American Cancer Society (through local chapters) provides information and counseling and can assist in obtaining financial assistance if needed. (800-ACS-2345; www.cancer.org.)

Hodgkin lymphoma

Hodgkin disease.

lymphoma

(lim-fo'ma ) ('mat-a) plural.lymphomaslymphomata [ lymph- + -oma]
A malignant neoplasm originating from lymphocytes. Common forms of lymphoma are listed in the subentries below. These include Hodgkin disease, mycosis fungoides, and non-Hodgkin lymphoma.lymphomatous ('mat-us), adjective See: Hodgkin disease

Staging

Staging of both Hodgkin and non-Hodgkin lymphoma is as follows: Stage I: involvement of a single lymph node or localized involvement. Stage II: Involvement of two or more lymph node regions on the same side of the diaphragm. Stage III: Involvement of several lymph node regions on both sides of the diaphragm. Stage IV: Involvement of extralymphatic tissue, such as the bone marrow.

anaplastic large cell lymphoma

Abbreviation: ALCL
A rare form of non-Hodgkin, T-cell lymphoma that may behave indolently when limited to the skin or may be more aggressive and spread to lymph nodes throughout the body.

body cavity lymphoma

Primary effusion lymphoma.

Burkitt lymphoma

See: Burkitt lymphoma
Enlarge picture
CUTANEOUS T-CELL LYMPHOMA: Raised reddish-purple plaque on the skin of the hip

cutaneous T-cell lymphoma

Abbreviation: CTCL.
A malignant non-Hodgkin lymphoma with a predilection for infiltrating the skin. In its earliest stages, it often is mistaken for a mild, chronic dermatitis because it appears as itchy macules and patches, often on the chest or trunk. Later, the lesions may thicken, become nodular, or spread throughout the entire surface of the skin, the internal organs, or the bloodstream.
See: illustration

follicular lymphoma

A B-cell, non-Hodgkin lymphoma found in adult and older patients. It results from a translocation of an oncogene from chromosome 14 to chromosome 18 [t(14; 18)]. Most instances of this lymphoma are indolent or slow growing.

hepatosplenic T-cell lymphoma

A rare, rapidly progressive lymphoma that develops in the liver, spleen, and bone marrow. It has been identified in patients taking immunosuppressive drugs for diseases such as inflammatory bowel disease.

Hodgkin lymphoma

See: Hodgkin, Thomas

Mediterranean lymphoma

Immunoproliferative small intestinal disease.
Enlarge picture
NON-HODGKIN LYMPHOMA: Bizarre-appearing lymphocytes revealing active mitosis (orig. mag. ×1000)

non-Hodgkin lymphoma

Abbreviation: NHL
Any of a group of malignant tumors of B or T lymphocytes. In 2008, the American Cancer Society estimated that about 66,100 Americans would be newly diagnosed with the disease. See: illustration; Hodgkin disease

Symptoms

Painless lymphadenopathy in two thirds of patients is the most frequent presenting symptom. Others have fever, night sweats, and loss of 10% or more of body weight in the 6 months before presenting with symptoms of infiltration into nonlymphoid tissue. Additional involvement is in peripheral areas such as epitrochlear nodes, the tonsillar area, and bone marrow. NHL is 50% more frequent in occurrence in men than in women of similar age. In most cases the cause of NHL is unknown, but patients who have received immunosuppressive agents have an over 100 times greater chance of developing NHL, probably because the immunosuppressive agents activate tumor viruses.

Treatment

Specific therapy depends on the type, grade, and stage of the lymphoma. Combination chemotherapies, bone marrow transplantation, radiation therapy, and photochemotherapy may be given, depending on the specific diagnosis.

primary effusion lymphoma

A non-Hodgkin B-cell lymphoma that typically arises in body cavities such as the pleural, peritoneal, or pericardial spaces. It is caused by Kaposisarcoma herpesvirus (human herpes virus 8) and is usually found in patients with advanced immune suppression.
Synonym: body cavity lymphoma

Hodgkin Lymphoma

DRG Category:824
Mean LOS:8.1 days
Description:SURGICAL: Lymphoma and Non-Acute Leukemia With Other O.R. Procedure With CC
DRG Category:841
Mean LOS:6.4 days
Description:MEDICAL: Lymphoma and Non-Acute Leukemia With CC

Hodgkin lymphoma is a group of neoplastic disorders characterized by painless, progressive enlargement of the lymph nodes, spleen, and other lymphoid tissue. The enlargement is caused by a proliferation of lymphocytes, histiocytes, eosinophils, and Reed-Sternberg giant cells, the cells that characterize Hodgkin lymphoma; their absence classifies a lymphoma as non-Hodgkin. Generally, the disease tends to begin within a single lymph node region and spreads to nodes in close proximity. Only late in the disease will widespread dissemination occur. It is a progressive and fatal disease if not treated but is one of the most curable neoplastic diseases with treatment. The World Health Organization has classified Hodgkin lymphoma in two ways: classic Hodgkin lymphoma (comprised of nodular sclerosis, mixed cellularity, lymphocyte depleted, and lymphocyte rich ) and nodular lymphocyte-predominant Hodgkin lymphoma.

Hodgkin lymphoma has a worldwide incidence of about 62,000 new cases a year and accounts for 0.7% of all cancers. Depending on the stage when the diagnosis is made, the 1-year survival rate with treatment is 93%, and the 5-year survival rate is 82%. At 15 years, the overall survival rate is 63%. Poorer survival rates occur in older people, those with bulky disease, and those with lymphocyte depletion.

Causes

The cause of Hodgkin lymphoma is unknown. Many researchers have suspected an infectious component. Some of the early symptoms include fever, chills, and leukocytosis, as if a viral infection were present. Gene fragments similar to those of a murine leukemia virus have been found in tissue of people with the disease. In particular, higher-than-usual levels of Epstein-Barr antibodies have been found in many patients, and a small increase in incidence has been found in people who have had Epstein-Barr–induced infectious mononucleosis. Some people who have reduced immune systems, such as those with AIDS and organ transplant patients, are also at a higher risk for Hodgkin lymphoma.

Genetic considerations

Twin studies and ethnic distribution patterns and occurrences of several affected persons in a family support a genetic predisposition for Hodgkin lymphoma. A human leukocyte antigen–linked locus has been proposed.

Gender, ethnic/racial, and life span considerations

Hodgkin lymphoma tends to strike in young adulthood from the ages of 15 to 38 and is more common in men than in women. When children get the disease, approximately 85% of the patients are male. There is also a bimodal incidence, with the first major peak being in young adults and the second peak later in life after age 50. Elderly people tend to have a more advanced disease at diagnosis and a worse prognosis for cure. The disease is more common among people with European ancestry than those with Asian or African ancestry.

Global health considerations

The global incidence of Hodgkin lymphoma is 1.2 per 100,000 males per year and 0.8 per 100,000 females per year. The incidence is two to four times higher in developed countries than in developing countries. Rates appear to be lower in Asian countries and the islands of the Pacific Ocean.

Assessment

History

Many patients present with asymptomatic peripheral adenopathy. Because there are numerous causes for enlarged lymph nodes, it is important to elicit information about recent infections, allergic reactions, and other events. In Hodgkin’s, the nodes tend to be cervical, supraclavicular, and mediastinal. About 40% of patients report fever, night sweats, and recent weight loss, collectively called B symptoms. Some patients report cough, chest pain, or dyspnea. Less commonly, they may report pruritus during any stage (Table 1). Because the B symptoms are necessary for staging, it is important to elicit that information in the history.

Staging for Hodgkin lymphoma
Table 1. Staging for Hodgkin lymphoma
STAGES AND SUBCLASSIFICATIONSDESCRIPTION5-YEAR SURVIVAL RATE BASED ON STAGE
Stage ILocalized to a single lymph node or nodal group90%–95%
Stage IIMore than one nodal group on the same side of the diaphragm90%–95%
Stage IIIMore than one nodal group on both sides of the diaphragm84%
Stage IVSpread to organs other than lymph nodes or spleenApproximately 65%
SubclassificationA: Asymptomatic
B: Fevers, weight loss, night sweats are present; symptoms suggest bulky disease and a poor prognosis (sometimes classified as “X” category)
E: Extralymphatic involvement such as stomach, small intestine
S: Spleen involvement

Physical examination

The most common presenting symptom is asymptomatic swelling of the lymph nodes above the diaphragm. During advanced phases of the disease, the patient may have edema of the face and neck, weight loss, and jaundice. Palpate all lymph node chains, including the submental, infraclavicular, epitrochlear, iliac, femoral, and popliteal nodes. Involved nodes are characteristically painless, firm, rubbery in consistency (unlike the rock-hard nodes of carcinoma), freely movable, and of varying size. Palpate the liver and spleen, which may be enlarged.

Psychosocial

The diagnosis of a neoplastic disorder in young adulthood is a devastating event for the patient and significant others. Rather than pursuing educational goals, job obligations, social interactions, or parenting responsibilities, the young adult is suddenly managing a potentially terminal disease. Although the disease is treatable in most cases, the patient needs to manage short- and long-term complications of therapy that may profoundly alter the patient’s body image. Infertility in young adults after treatment may affect the patient’s view of herself or himself and the long-term potential for the desired role of parenthood.

Diagnostic highlights

TestNormal ResultAbnormality With ConditionExplanation
Lymph node biopsy or bone marrow biopsyNormal cellsPositive for Hodgkin’s lymphoma cells (Reed-Sternberg giant cells)Determines extent of disease and allows for staging of disease; bone marrow biopsy is generally done only for patients with anemia or fever and night sweats
Computed tomography (CT) scan or magnetic resonance imaging of chest, abdomen, and pelvisNormal structuresSpread of Hodgkin’s lymphoma into organs and body cavitiesAssists with staging; common sites of extralymphatic involvement include spleen, stomach, small intestine; combined with lymphangiography, can predict nodal involvement in 90% of cases
Lymphangiography, a radiographic test of lymphatic vessels and nodes; radiopaque iodine contrast medium is injected into lymphatics of foot or handNormal lymphatic systemIdentification of structural abnormalities or tumor involvementTest has been replaced in many situations by CT scanning but may still be used for staging; not usually performed in children

Other Tests: Complete blood cell count, chest x-ray, erythrocyte sedimentation rate; tests for liver and renal function, including lactate dehydrogenase, alkaline phosphatase, blood urea nitrogen, creatinine

Primary nursing diagnosis

Diagnosis

Risk for infection related to impaired primary and secondary defenses

Outcomes

Immune status; Knowledge: Infection control; Risk control; Risk detection; Nutritional status; Tissue integrity: Skin and mucous membranes; Treatment behavior: Illness or injury

Interventions

Infection control; Infection protection; Surveillance; Nutritional management; Medication management; Teaching: Disease process

Planning and implementation

Collaborative

Treatment begins with accurate classification and staging. Clinical staging is determined by initial biopsy, history, physical examination, and radiological findings. Pathological staging involves a more extensive surgical assessment of possible sites for spread. Owing to continued improvement in radiological staging, a staging laparotomy (thorough abdominal exploration, splenectomy, liver biopsy, bone marrow biopsy, and multiple lymph node samplings) is performed infrequently.

In general, radiation is used for early, less extensive disease. A combination of radiation and chemotherapy is used for stages IIB, IIIA, and B. Combination chemotherapy with drugs such as doxorubicin, bleomycin, vinblastine, and dacarbazine (ABVD) is used for stage IV (see Pharmacologic Highlights). External beam radiation is the most effective single agent in the treatment of Hodgkin lymphoma and may be given after three to four courses of chemotherapy. Stages I and IIA Hodgkin lymphoma are routinely treated with external beam radiation therapy. Mantle therapy (radiation to the chest wall, mediastinum, axilla, and neck—the region known as the mantle field) is done for supradiaphragmatic sites. Radiation-protective shields are used to block irradiation to unaffected areas. These shields are custom fit for each patient according to his or her physical configurations. Surgery is not used as a treatment modality in Hodgkin’s except in the role of staging. A dietary consultation may be needed to help the patient maintain weight and to help support healing.

If the disease does not respond to standard treatment, bone marrow transplantation may be offered, either as part of a clinical trial or outside of a clinical trial. The patient’s own bone marrow is removed and stored. Then very high doses of chemotherapy, sometimes in combination with radiation therapy, are administered to eradicate the cancer. High doses also destroy bone marrow. The stored marrow is administered intravenously to the patient, and bone marrow cells enter the bloodstream and return to the bone. The transplanted marrow produces new red and white blood cells. In another type of transplant, peripheral blood stem cell transplant, only the stem cells (immature cells from which all blood cells develop) are removed and the rest of the blood is returned to the body. Stem cells are then frozen until they are returned to the patient after treatment is finished.

Pharmacologic highlights

General Comments: Typically, chemotherapy is given in six or more cycles of treatment in combination with radiotherapy. Common side effects are alopecia, nausea, vomiting, fatigue, myelosuppression, and stomatitis. Patients who are receiving chemotherapy are administered antinausea drugs, antiemetics, and pain medicines as needed to help control adverse experiences. Note that elderly patients are at particular risk for developing toxicity from chemotherapy and need to be monitored carefully.

Medication or Drug ClassDosageDescriptionRationale
ChemotherapyVaries with drugCommon examples are ABVD (adriamycin, bleomycin, vinblastine, dacarbazine), Stanford V (doxorubicin, vinblastine, mustard, bleomycin, vincristine, etoposide, prednisone), and BEACOPP (bleomycin, etoposide, doxorubicin, cyclophosphamide, vincristine, procarbazine, prednisone)Chemotherapy is used depending on early or advanced disease

Other Drugs: Stanford V (doxorubicin, vinblastine, mustard, bleomycin, vincristine, etoposide, and prednisone) is another drug regimen. The drugs are administered weekly, alternating the myelosuppressive and nonmyelosuppressive agents for 12 weeks, followed by radiation therapy.

Independent

The primary nursing roles are to maintain comfort, protect the patient from infection, provide teaching and support about the complications of the treatment, and give emotional support. During mantle irradiation, the patient may suffer from a variety of uncomfortable or painful conditions. Dry mouth, loss of taste, dysphagia, nausea, and vomiting can be managed with frequent mouth care. Manage skin irritation and redness. Encourage the patient to avoid applying lotions, perfumes, deodorants, and powder to the treatment area. Explain that the skin must be protected from sunlight and extreme cold. Before starting treatments, arrange for the patient to have a wig, scarf, or hat to cover any hair loss, which occurs primarily at the nape of the neck. Explain to the patient that pneumonitis and hypothyroidism may occur; explain the signs and symptoms of each and when to notify the physician. During inverted-Y irradiation, nausea, vomiting, anorexia, diarrhea, and malaise require nursing management.

If the patient develops bone marrow suppression during hospitalization, make sure all staff and visitors use good hand-washing techniques. Do not assign a nurse who is caring for patients with infections. Encourage staff and visitors with infections to avoid all contact with the patient. If the patient receives chemotherapy, the side effects are equally uncomfortable. In addition to many of the symptoms that occur in response to radiation therapy (gastrointestinal symptoms, oral lesions, hair loss, bone marrow depression), the patient may develop joint pain, fever, fluid retention, and a labile emotional state (euphoria or depression) that need specific interventions based on their incidence and severity.

The disease presents severe emotional stressors to the patient and her or his significant others. The complexity of the diagnostic and staging process may make the patient feel lost in a crowd of specialists. It is important for the nurse to provide supportive continuity. Patience and repeated explanations are needed. Provide the patient with information about support groups, and refer the patient to either a clinical nurse specialist, support groups associated with the American or Canadian Cancer Society, or counselors.

Evidence-Based Practice and Health Policy

Milano, M.T., Li, H., Gail, M.H., Constine, L.S., & Travis, L.B. (2010). Long-term survival among patients with Hodgkin's lymphoma who developed breast cancer: A population-based study. Journal of Clinical Oncology, 28(34), 5088–5096.

  • The risks of developing other cancers are increased in patients treated with radiotherapy for Hodgkin lymphoma.
  • In a study that compared 298 Hodgkin lymphoma survivors with breast cancer to 405,223 women with a single or primary breast cancer diagnosis, mortality rate was doubled among the Hodgkin lymphoma survivors (p < 0.001).
  • The 15-year survival rate for localized breast cancer was 48% among Hodgkin lymphoma survivors compared to 69% in the women with a primary breast cancer diagnosis and 33% compared to 43% for regional or distant breast cancer, respectively (p < 0.0001).

Documentation guidelines

  • Response to staging: Emotional and physical response to diagnostic testing, healing of incisions, signs of ineffective coping, response to diagnosis, ability to participate in planning treatment options, response of significant others
  • Response to treatment: Effects of chemotherapy or radiation therapy, or both; response to treatment of symptoms, presence of complications (weight loss, infection, skin irritation)
  • Emotional state: Effectiveness of coping, presence of depression, interest in group support or counseling, referrals made

Discharge and home healthcare guidelines

Although they are cured of the disease, patients who survive Hodgkin lymphoma continue to have immune defects that persist throughout life. Defects include transiently depressed antibody production, decreased polymorphonuclear chemotaxis, decreased antigen-induced T-cell proliferation, and changes in delayed hypersensitivity. Coupled with the sometimes lingering after-effects of radiation and chemotherapy, the patient needs to maintain infection vigilance even after remission is obtained. Teach the patient lifelong strategies to avoid infection.

Patients may have other complications for up to 25 years after mantle radiation therapy, including hypothyroidism, Graves’ disease, and thyroid cancer. Irradiation can also cause pulmonary and pericardial fibrosis and coronary artery changes, and it may increase the risk for the development of solid tumors such as lung cancer, breast cancer, and others. Explain the presenting symptoms of the disorder, provide written information for the patient, and encourage yearly physicals to maintain follow-up. Because infertility may be a complication of chemotherapy, men may want to think of sperm banking before treatments, although many have sperm dysfunction at diagnosis.

References in periodicals archive ?
The most common pattern diffuse large B-cell Non Hodgkin lymphoma infiltraton was bone marrow followed by interstitial tissue.
Conclusion: EBV is a major risk factor for Mixed Cellularity and Nodular Sclerosis subtypes of Hodgkin Lymphoma in KPK Province of Pakistan.
Single-arm study of brentuximab vedotin in patients with relapsed or refractory Hodgkin lymphoma (RRHL) who are ineligible for stem cell transplantation (SCT) or multi-agent chemotherapy.
Impression given as suggestive of Hodgkin Lymphoma (Figure 3).
The immunophenotype did not support classical Hodgkin lymphoma, as the RS-like cells expressed CD45, CD20, and BCL-6 and were negative for fascin.
Coverage of the Hodgkin Lymphoma pipeline on the basis of route of administration and molecule type.
This finding effectively excludes nodular sclerosis Hodgkin lymphoma.
Shlansky-Goldberg et al described the case of nodular sclerosing Hodgkin lymphoma.
ODAC will review the BLA for the treatment of relapsed or refractory Hodgkin lymphoma during the morning session and the BLA for the treatment of relapsed or refractory systemic anaplastic large cell lymphoma (ALCL) during the afternoon session.