Clinical characteristics and outcome of pediatric patients with stage IV Hodgkin lymphoma.

BACKGROUND AND OBJECTIVES: While treatment outcomes for patients with Hodgkin lymphoma (HL) have improved remarkably, patients with disseminated disease still have a poorer outcome. Stage IV HL is often reported with other 'advanced stage' categories, confusing the specific contribution of disease dissemination to the outcome. This single-institution report looks at characteristics and outcomes of this specific category. PATIENTS AND METHODS: The medical records of pediatric HL patients (< 14 years) from 1975 through 2003 were retrospectively reviewed and the data analyzed. RESULTS: Stage IV patients (n = 67) had more poor-risk characteristics than patients in stages I-III (n = 300) (B symptoms 86.6% vs. 19.3%, bulky disease 57.6% vs. 45.5% and mediastinal mass 77.6% vs. 29.7%; P < .001 for all characteristics). The liver was the most common extralymphatic site (in 51.5% of patients with stage IV disease. Stage IV patients received chemotherapy (CT) alone (n = 55) or combined modality therapy (CMT) (n = 12). Fifty-four patients (80.6%) achieved complete remission, 2 (3%) partial remission, 10 (14.9%) had progressive disease and 1 was lost to follow up. Overall survival was 79.4% and event-free survival (EFS) was 63.9% at 5 years. There was a non-significant benefit for CMT (OS = 91.7% v. 77.1%, P = .3; EFS = 70.7% v. 62.7%, P = .3). Ten of 12 relapsed and only 1 of 10 progressive disease patients were salvaged. On multivariate analysis, failure to achieve complete remission with CT was associated with a poorer outcome. CONCLUSION: Stage IV disease is associated with poor risk features and confers a worse outcome than stage I-III disease. Achievement of complete remission with CT is an important prognostic feature. Slow responders may require novel and/or aggressive therapy to achieve complete remission.

Hodgkin lymphoma in very young children: Clinical characteristics and outcome of treatment.

In developed nations, Hodgkin lymphoma (HL) is rare in <5-year olds and represent a minority in developing countries. Little is reported about the biology and behavior of these very young patients compared with older children. 18.75% of our pediatric HL patients (0 - 14 years) were <5 years at diagnosis. This group had more boys, similar incidence of B-symptoms and stage distribution, less mediastinal involvement and bulky disease, and more mixed cellularity subtype than older children. Treatment included chemotherapy (CT; n = 55), combined modality therapy (CMT; n = 12) and XRT only (n = 2). Ten-year EFS and OS was 81.5% and 90.4%, respectively, versus 75.5% and 90.5% for older children (p > 0.5). A trend toward better survival was seen with CMT, using very LD-XRT, than with CT (OS 100% vs. 86.4%[p = 0.3]; EFS 90.9% vs. 81.0%[p = 0.4]). Although CT could be effective in a subset of LR patients, LD-XRT may be needed to effectively treat most of these patients. This dose reduction may reduce XRT-related toxicity, which can be significant in very young children.