Chapter 105. Malignancies of Lymphoid Cells (Part 23)

The diagnosis of Hodgkins disease is established by review of an adequate biopsy specimen by an expert hematopathologist. In the United States, most patients have nodular sclerosing Hodgkins disease, with a minority of patients having mixed-cellularity Hodgkins disease. Lymphocyte-predominant and lymphocyte-depleted Hodgkins disease are rare. Mixed-cellularity Hodgkins disease or lymphocyte-depletion Hodgkins disease are seen more frequently in patients infected by HIV (Fig. 105-11). The differential diagnosis of a lymph node biopsy suspicious for Hodgkins disease includes inflammatory processes, mononucleosis, non-Hodgkins lymphoma, phenytoin-induced adenopathy, and nonlymphomatous malignancies.Figure 105-11Mixed cellularity Hodgkins disease. A Reed-Sternberg cell is present near the center of...
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Chapter 105. Malignancies of Lymphoid Cells (Part 23) Chapter 105. Malignancies of Lymphoid Cells (Part 23) The diagnosis of Hodgkins disease is established by review of an adequatebiopsy specimen by an expert hematopathologist. In the United States, mostpatients have nodular sclerosing Hodgkins disease, with a minority of patientshaving mixed-cellularity Hodgkins disease. Lymphocyte-predominant andlymphocyte-depleted Hodgkins disease are rare. Mixed-cellularity Hodgkinsdisease or lymphocyte-depletion Hodgkins disease are seen more frequently inpatients infected by HIV (Fig. 105-11). The differential diagnosis of a lymph nodebiopsy suspicious for Hodgkins disease includes inflammatory processes,mononucleosis, non-Hodgkins lymphoma, phenytoin-induced adenopathy, andnonlymphomatous malignancies. Figure 105-11 Mixed cellularity Hodgkins disease. A Reed-Sternberg cell is presentnear the center of the field; a large cell with a bilobed nucleus and prominentnucleoli giving an owls eyes appearance. The majority of the cells are normallymphocytes, neutrophils, and eosinophils that form a pleiomorphic cellularinfiltrate. The staging evaluation for a patient with Hodgkins disease would typicallyinclude a careful history and physical examination; complete blood count;erythrocyte sedimentation rate; serum chemistry studies including LDH; chestradiograph; CT scan of the chest, abdomen, and pelvis; and bone marrow biopsy.Many patients would also have a PET scan or a gallium scan. Although rarelyutilized, a bipedal lymphangiogram can be helpful. PET and gallium scans aremost useful to document remission. Staging laparotomies were once popular formost patients with Hodgkins disease but are now done rarely because of anincreased reliance on systemic rather than local therapy. Classical Hodgkins Disease: Treatment Patients with localized Hodgkins disease are cured >90% of the time. Inpatients with good prognostic factors, extended-field radiotherapy has a high curerate. Increasingly, patients with all stages of Hodgkins disease are treated initiallywith chemotherapy. Patients with localized or good-prognosis disease receive abrief course of chemotherapy followed by radiotherapy to sites of nodeinvolvement. Patients with more extensive disease or those with B symptomsreceive a complete course of chemotherapy. The most popular chemotherapyregimens used in Hodgkins disease include doxorubicin, bleomycin, vinblastine,and dacarbazine (ABVD) and mechlorethamine, vincristine, procarbazine, andprednisone (MOPP), or combinations of the drugs in these two regimens. Today,most patients in the United States receive ABVD, but a weekly chemotherapyregimen administered for 12 weeks called Stanford V is becoming increasinglypopular, but includes radiation therapy, which has been associated with life-threatening late toxicities such as premature coronary artery disease and secondsolid tumors. In Europe a high-dose regimen called BEACOPP incorporatingalkylating agents has become popular and might have a better response rate invery high risk patients. Long-term disease-free survival in patients with advanceddisease can be achieved in >75% of patients who lack systemic symptoms and in60–70% of patients with systemic symptoms. Patients who relapse after primary therapy of Hodgkins disease canfrequently still be cured. Patients who relapse after initial treatment only withradiotherapy have an excellent outcome when treated with chemotherapy. Patientswho relapse after an effective chemotherapy regimen are usually not curable withsubsequent chemotherapy administered at standard doses. However, patients witha long initial remission can be an exception to this rule. Autologous bone marrowtransplantation can cure half of patients who fail effective chemotherapy regimens. Because of the very high cure rate in patients with Hodgkins disease, long-term complications have become a major focus for clinical research. In fact, insome series of patients with early-stage disease, more patients died from latecomplications of therapy than from Hodgkins disease itself. This is particularlytrue in patients with localized disease. The most serious late side effects includesecond malignancies and cardiac injury. Patients are at risk for the development ofacute leukemia in the first 10 years after treatment with combinationchemotherapy regimens that contain alkylating agents plus radiation therapy. Therisk for development of acute leukemia appears to be greater after MOPP-likeregimens than with ABVD. The risk of development of acute leukemia aftertreatment for Hodgkins disease is also related to the number of exposures topotentially leukemogenic agents (i.e., ...