BACKGROUND: 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. PROCEDURE: 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. RESULTS: 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. CONCLUSIONS: Curative treatment of NHL in the presence of primary ID is possible and should be attempted. Copyright 1999 Wiley-Liss, Inc.
BACKGROUND: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. PROCEDURE: 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. RESULTS: 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. CONCLUSIONS: Curative treatment of NHL in the presence of primary ID is possible and should be attempted. Copyright 1999 Wiley-Liss, Inc.
Authors: Andishe Attarbaschi; Elisa Carraro; Oussama Abla; Shlomit Barzilai-Birenboim; Simon Bomken; Laurence Brugieres; Eva Bubanska; Birgit Burkhardt; Alan K S Chiang; Monika Csoka; Alina Fedorova; Janez Jazbec; Edita Kabickova; Zdenka Krenova; Jelena Lazic; Jan Loeffen; Georg Mann; Felix Niggli; Natalia Miakova; Tomoo Osumi; Leila Ronceray; Anne Uyttebroeck; Denise Williams; Wilhelm Woessmann; Grazyna Wrobel; Marta Pillon Journal: Haematologica Date: 2016-08-11 Impact factor: 9.941
Authors: Krystyna H Chrzanowska; Hanna Gregorek; Bożenna Dembowska-Bagińska; Maria A Kalina; Martin Digweed Journal: Orphanet J Rare Dis Date: 2012-02-28 Impact factor: 4.123
Authors: Simon Bomken; Jutte van der Werff Ten Bosch; Andishe Attarbaschi; Chris M Bacon; Arndt Borkhardt; Kaan Boztug; Ute Fischer; Fabian Hauck; Roland P Kuiper; Tim Lammens; Jan Loeffen; Bénédicte Neven; Qiang Pan-Hammarström; Isabella Quinti; Markus G Seidel; Klaus Warnatz; Claudia Wehr; Arjan C Lankester; Andrew R Gennery Journal: Front Immunol Date: 2018-12-12 Impact factor: 7.561