Improvement of survival in patients with primary cutaneous diffuse large B-cell lymphoma, leg type, in France

Abstract

IMPORTANCE: Primary cutaneous diffuse large B-cell lymphoma, leg type (PCDLBCL-LT), occurs in elderly patients and has been considered as a lymphoma with a poor prognosis, with estimated 5-year specific survival rates of approximately 50%. The hypothesis of an improvement in prognosis over time has not been studied. OBJECTIVES: To evaluate this hypothesis in a large series of patients and investigate factors associated with prognosis as well as improvement in the prognosis. DESIGN, SETTING, AND PARTICIPANTS: A retrospective multicenter studywas conducted including dermatology departments belonging to the French Study Group on Cutaneous Lymphoma. Participants were 115 patients with PCDLBCL-LT diagnosed between 1988 and 2003 (period 1) or between 2004 and 2010 (period 2). MAIN OUTCOMES AND MEASURES: Age, sex, period of diagnosis, number of skin lesions, tumor stage, tumor location (leg vs nonleg), lactate dehydrogenase level, type of therapy (with or without a combination of rituximab and polychemotherapy [PCT]), and outcome were recorded. Baseline characteristics and outcome were compared according to period of diagnosis and type of therapy. Prognosis factors were identified by univariate and multivariate survival analyses. RESULTS: The mean age of the patients was 76.9 years, and 47%of the patients were older than 80 years. The 3- and 5-year specific survival rates improved between period 1 and period 2, from 55%to 74%and from 46%to 66%, respectively (P =.01). Patients had similar baseline characteristics during both periods, but rituximab-PCT regimens were administered to 88.5%of the patients in period 2 vs 16.7%in period 1 (P