Résumé
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 repported 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. The medical records of pediatric HL patients [<14 years] from 1975 through 2003 were retrospectively reviewed and the data analyzed. 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 diseease. 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. 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