Therapy for non-Hodgkin lymphoma in children with primary immunodeficiency: Analysis of 19 patients from the BFM trials

Abstract

Non-Hodgkin lymphomas (NHL) represent an important complication of primary immunodeficiency (ID), posing new therapeutic challenges in this patient population. This study analyzes clinical data and therapy results of pediatric patients with primary ID and NHL in three consecutive NHL-BFM trials. Retrospective analysis of children with primary ID and NHL, treated according to protocol NHL-BFM, was performed regarding clinical presentation, diagnostic features, therapy, and outcome. From October, 1986, to April, 1997, 19 of 1,413 newly diagnosed patients with NHL were registered as suffering from primary ID. Age at diagnosis of NHL was lower in patients with ID. Six patients suffered from humoral ID, 13 patients from combined ID (ataxia teleangiectasia n = 3; Nijmegen breakage syndrome n = 4; PNP deficiency n = 1; IL2 receptor defect n = 1, other combined ID n = 4). Thirteen lymphomas were of B-cell and six of T-cell-lineage. Four of thirteen patients with combined ID were diagnosed with T-NHL, nine with B-NHL. Two of six patients with humoral ID presented with T-NHL and four with B-NHL. NHL entities differed significantly between ID and non-ID patients (P < or = 0.01): centroblastic and immunoblastic lymphomas (31.6% vs. 8.1%), anaplastic large cell lymphoma (26.3% vs. 10.7%), Burkitt lymphoma and B-ALL (21% vs. 47. 8%). Seventeen patients received polychemotherapy. Therapy-related toxicity was increased in ID- compared to non-ID-patients. Three patients died of sepsis; three died of tumor progression; one patient relapsed; one died of BMT-related toxicity; one died of second malignancy. Ten patients are in first continuous remission after a median follow-up of 4 years. Curative treatment of NHL in the presence of primary ID is possible and should be attempted.