Malignancies in Primary Immunodeficiencies: A Single Center Experience

作者: Turkan Patiroglu , H. Haluk Akar , Ekrem Unal , M. Akif Ozdemir , Musa Karakukcu

DOI: 10.1089/PED.2014.0453

关键词:

摘要: Background and Aim: Primary immunodeficiencies (PIDs) are genetic disorders that predispose patients to recurrent infections, autoimmunity, malignancy. This study aimed investigate the clinical histopathological characteristics of malignancies in PIDs. Materials Methods: Herein, 11 (1 boy, 10 girls) with PIDs presented dating between 1996 2014. The were analyzed retrospectively. Results: In this 18-year period, 450 diagnosed as having PIDs, there cancer cases associated age ranged from 3 20 years a mean 10.5±6 at time diagnosis malignancies. Lymphomas observed 73% (8/11) patients. Non-Hodgkin's lymphomas (NHL) Hodgkin's (HL) seen 63% (5/8) 37% (3/8) patients, respectively. five (45%; 5/11) was diffuse large B-cell lymphoma (DLBCL), three them T-cell defects unknown etiolog...

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