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New Treatments for Hodgkins Disease

The incidence of Hodgkin's disease exhibits a characteristic bimodal distribution in regard to age. In the United States and other industrialized countries, the early peak occurs in the mid to late 20s and the second peak after the age of 50. In developing countries, the early peak occurs before adolescence. Epidemiologic studies demonstrate three distinct forms of Hodgkin's disease, the characteristics of which suggest different causes: a childhood form, in patients 14 years or younger; a young adult form, in patients 15 to 34 years; and an older adult form.

Clustering of Hodgkin's cases within families or races has been attributed to genetic predisposition to the disease, or to common exposure to a causal agent. Studies of familial Hodgkin's disease have indicated increased association with specific human leukocyte antigens (HLAs). Many investigators have observed concordance of Hodgkin's disease.

Several epidemiologic studies have suggested that infectious agents, including herpes virus 6, cytomegalovirus, and Epstein-Barr virus (EBV), may be involved in the transmission of Hodgkin's disease. Enhanced activation of EBV, suggested by the high EBV antibody titers observed in many patients, and in situ hybridization evidence of EBV genomes in Reed-Sternberg cells, support the hypothesis that EBV may play a role in the pathogenesis.

CLINICAL PRESENTATION

Asymptomatic cervical or supraclavicular lymphadenopathy, which may fluctuate over time, is the most common presentation of Hodgkin's disease in children. Two thirds of patients will also have mediastinal adenopathy, which may produce symptoms of tracheal or bronchial compression. Infrequently, axillary or inguinal lymphadenopathy is the first presenting sign.

The association of Hodgkin's disease with idiopathic thrombocytopenic purpura (ITP) has been observed by many investigators, and the prognosis for response to ITP therapy is related to the status of the underlying Hodgkin's disease. ITP that develops during remission of Hodgkin's.

STAGING

Hodgkin's disease spreads contiguously to adjacent lymph nodes until late in the course of the disease. The anatomically based Ann Arbor staging system, adopted in 1971, assigns stage based on the number of sites of lymph node involvement, the presence of extranodal disease, and a history of B symptoms. Patients are classified as A if asymptomatic and as B if symptoms of a temperature of over 38C for 3 consecutive days, drenching night sweats.

If the treatment plan prescribes radiation therapy only, precise anatomic staging is required for the design of radiation fields. However, if chemotherapy is planned, histologic confirmation.

TREATMENT

Optimal therapy planning for pediatric Hodgkin's disease requires a multidisciplinary approach from the time of diagnosis. Factors influencing treatment decisions are the age and physical maturity of the patient, the disease stage and bulk, and potential treatment sequelae.

Treatment Results

Early-Stage and Favorable Disease

High-dose radiation therapy is reserved for early-stage and nonbulky Hodgkin's disease in physically mature patients. Most commonly this involves treatment to extended fields. Treatment results using full-dose (35-44 Gy) radiation therapy in pathologic stage I or IIA children has a 5-year DFS rates ranging from 70% to 86% at single institutions to 30% to 60% in multi-institutional trials. Multiple centers have reported excellent cure rates for favorable, limited-stage Hodgkin's disease by using six courses of combination chemotherapy (nitrogen mustard, Oncovin (vincristine), procarbazine, and prednisone [MOPP], or Adriamycin (doxorubicin), bleomycin, vinblastine, and dacarbazine [ABVD].

Some groups have reported treatment results with chemotherapy alone using MOPP and similar therapies containing alkylating agents for early-stage Hodgkin's disease.