Hodgkin's disease
Hodgkin's disease: Excerpt from Professional Guide to Diseases (Eighth Edition)
Hodgkin's disease is a neoplastic disease characterized by painless, progressive enlargement of lymph nodes, spleen, and other lymphoid tissue resulting from proliferation of lymphocytes, histiocytes, eosinophils, and Reed-Sternberg giant cells. The latter cells are its special histologic feature. Untreated, Hodgkin's disease follows a variable but relentlessly progressive and ultimately fatal course. However, recent advances in therapy make Hodgkin's disease potentially curable, even in advanced stages; appropriate treatment yields a 5-year survival rate in approximately 80% of patients.
Causes and incidence
Although the cause of Hodgkin's disease is unknown, a viral etiology is suspected, with the Epstein-Barr virus as a leading candidate. The disease is most common in young adults, with a higher incidence in males than in females. It occurs in all races but is slightly more common in whites. Its incidence peaks in two age-groups: 15 to 38 and after age 50 — except in Japan, where it occurs exclusively among people older than 50.
Signs and symptoms
The first sign of Hodgkin's disease is usually a painless swelling of one of the cervical lymph nodes (but sometimes the axillary, mediastinal, or inguinal lymph nodes), occasionally in a patient who gives a history of recent upper respiratory infection. In older patients, the first signs and symptoms may be nonspecific — persistent fever, night sweats, fatigue, weight loss, and malaise. Rarely, if the mediastinum is initially involved, Hodgkin's may produce respiratory symptoms.
Another early and characteristic indication of Hodgkin's disease is pruritus, which, although mild at first, becomes acute as the disease progresses. Other symptoms depend on the degree and location of systemic involvement.
Lymph nodes may enlarge rapidly, producing pain and obstruction, or enlarge slowly and painlessly for months or years. It isn't unusual to see the lymph nodes “wax and wane,'' but they usually don't return to normal. Sooner or later, most patients develop systemic manifestations, including enlargement of retroperitoneal nodes and nodular infiltrations of the spleen, the liver, and bones. At this late stage other symptoms include edema of the face and neck, progressive anemia, possible jaundice, nerve pain, and increased susceptibility to infection.
Diagnosis
Diagnostic measures for confirming Hodgkin's disease include a thorough medical history and a complete physical examination, followed by a lymph node biopsy checking for Reed-Sternberg's abnormal histiocyte proliferation and nodular fibrosis and necrosis. (See Reed-Sternberg cells.)
Other appropriate diagnostic tests include bone marrow, liver, mediastinal, lymph node, and spleen biopsies and routine chest X-ray, abdominal computed tomography scan, positron emission tomography, lung scan, bone scan, and lymphangiography to detect lymph node or organ involvement. Laparoscopy and lymph node biopsy are performed to complete staging.
Hematologic tests show mild to severe normocytic anemia; normochromic anemia (in 50%); elevated, normal, or reduced white blood cell count and differential showing any combination of neutrophilia, lymphocytopenia, monocytosis, and eosinophilia. Elevated serum alkaline phosphatase indicates liver or bone involvement.
The same diagnostic tests are also used for staging. A staging laparotomy is necessary for patients younger than age 55 or without obvious stage III or stage IV disease, lymphocyte predominance subtype histology, or medical contraindications. Diagnosis must rule out other disorders that also enlarge the lymph nodes.
Treatment
Appropriate therapy (chemotherapy or radiation, or both, varying with the stage of the disease) depends on careful physical examination with accurate histologic interpretation and proper clinical staging. (See Staging Hodgkin's disease.) Correct and timely treatment allows longer survival and even induces an apparent cure in many patients. Radiation therapy is used alone for stages I and II and in combination with chemotherapy for stage III. Chemotherapy is used for stage IV, sometimes inducing a complete remission. The well-known MOPP protocol (mechlorethamine, vincristine [Oncovin], procarbazine, and prednisone) was the first to provide significant cures to patients with generalized Hodgkin's; another useful combination is ABVD (doxorubicin [Adriamycin], bleomycin, vinblastine, and dacarbazine). Another chemotherapy regimen — bleomycin, etoposide, cyclophosphamide, vincristine, procarbazine, and prednisone — has also shown promise in advanced Hodgkin's disease. Treatment with these drugs may require concomitant antiemetics, sedatives, or antidiarrheals to combat GI adverse effects.
New treatments include high-dose chemotherapeutic agents with autologous bone marrow transplantation or autologous peripheral blood stem cell transfusions. Biotherapy alone hasn't proven effective.
Special considerations
Because many patients with Hodgkin's disease receive radiation or chemotherapy as outpatients, tell the patient to observe the following precautions:
❑Watch for and promptly report adverse effects of radiation and chemotherapy (particularly anorexia, nausea, vomiting, diarrhea, fever, and bleeding).
❑Minimize adverse effects of radiation therapy by maintaining good nutrition (aided by eating small, frequent meals of his favorite foods), drinking plenty of fluids, pacing his activities to counteract therapy-induced fatigue, and keeping the skin in irradiated areas dry.
❑Control pain and bleeding of stomatitis by using a soft toothbrush, cotton swab, or anesthetic mouthwash such as viscous lidocaine (as prescribed), by applying petroleum jelly to his lips, and by avoiding astringent mouthwashes.
❑If a female patient is of childbearing age, advise her to delay pregnancy until prolonged remission because radiation and chemotherapy can cause genetic mutations and spontaneous abortions.
❑Because the patient with Hodgkin's disease has usually been healthy up until this point, he's likely to be especially distressed. Provide emotional support and offer appropriate reassurance. Ease the patient's anxiety by sharing your optimism about his prognosis.
❑Make sure both the patient and his family know that the local chapter of the American Cancer Society is available for information, financial assistance, and supportive counseling.
❑The development of further malignancies, such as acute myeloid leukemia and myelodysplastic syndrome, in patients successfully treated for Hodgkin's disease is a concern. Patients must be educated as to the importance of long-term follow-up care following completion of treatment.
Pictures
Book Source Details
- Book Title: Professional Guide to Diseases (Eighth Edition)
- Author(s): Springhouse
- Year of Publication: 2005
- Copyright Details: Professional Guide to Diseases (Eighth Edition), Copyright © 2005 Lippincott Williams & Wilkins.
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Copyright notice for book excerpts: Copyright © 2008 Lippincott Williams & Wilkins. All rights reserved.
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