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Download 1 - European Society for Immunodeficiencies
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Transcript
Indication and outcome of hematopoietic stem cell transplantation in primary immunodeficiency with predominant hypogammaglobulinemia – a retrospective study Objective: The proposed study aims at evaluating patient numbers, outcome and immune reconstitution after hematopoietic stem cell transplantation (HSCT) in patients with primary immunodeficiency with predominant hypogammaglobulinemia / antibody deficiency (PAD) in a retrospective multi-centric survey. PAD is the most common inborn immunodeficiency in adults. Patients suffer from severe, recurrent infections that can be associated with other co-morbidities such as autoimmunity, granuloma, lymphoproliferation and malignancies. The most common malignancy in adult PAD is lymphoma occurring in ~4-8% of patients. Hematopoietic stem cell transplantation (HSCT) has been used for single PAD patients with secondary malignancy or suspected combined immunodeficiency, however, its risks and benefits for PAD patients and the circumstances under which HSCT can cure the immunodeficiency have not been assessed systematically. Eligibility: All available patients (≥ 2 years at transplantation) with primary immunodeficiency with predominant antibody deficiency having undergone HSCT for any reason until March 2013. Predominant antibody deficiency is defined as severe reduction in at least 2 serum immunoglobulin isotypes with normal or low number of B cells (IUIS criteria table 3.2) or as a a severe reduction in all serum immunoglobulin isotypes with profoundly decreased or absent B cells (IUIS criteria table 3.1). The requested information is part of the dataset of the EBMT (European Group for Blood and Marrow Transplantation) and the ESID (European Society for Immunodeficiencies) registries. Patients have to consent to either data collection via ESID or EBMT registry. Endpoints: In this study we will combine transplant related data and data specific for immunodeficient patients (major infection frequency, lymphocyte counts, vaccination response, immunoglobulin levels pre- and post-transplant) in order to assess number, indication, mortality, morbidity and immune reconstitution after HSCT in PAD patients. Adult and pediatric patients will be compared with regard to reconstitution of the adaptive immune system. Duration of the data collection period: 3-6 months Contact: Prof. Dr. Klaus Warnatz Prof. Dr. Jürgen Finke Dr. Claudia Wehr Dr. Marta Rizzi [email protected] [email protected] [email protected] [email protected] Centre for Chronic Immunodeficiency and Department of Hematology and Oncolgy University Medical Centre Freiburg Breisacher Straße 117 – 2nd floor 79106 Freiburg Germany Tel: +49 (0)761 270-77640 Fax: +49 (0)761 270-77600