Immune reconstitution in patients with Fanconi anemia after allogeneic bone marrow transplantation |
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| Institution: | 1. Flow Cytometry Service Core, Clinics Hospital, Federal University of Paraná, Curitiba, PR, Brazil;2. Pediatric Bone Marrow Transplantation Division, Federal University of Paraná, Curitiba, Brazil;3. Regional Blood Center of Ribeirão Preto, Ribeirão Preto, Brazil;4. Cancer Research Center (IBMCC-CSIC/USAL), Department of Medicine, Cytometry Service and IBSAL, University of Salamanca, Salamanca, Spain;5. Hematology Division, Federal University of Paraná, Curitiba, Brazil;1. Peking University People''s Hospital and Peking University Institute of Hematology, Beijing Key Laboratory of Hematopoietic Stem Cell Transplantation, Beijing, China;2. Peking-Tsinghua Center for Life Sciences, Beijing, China;1. Laboratory of Experimental Hematology, Vaccine & Infectious Disease Institute (Vaxinfectio), Faculty of Medicine and Health Sciences, University of Antwerp, Wilrijk, Belgium;2. Department of Neurology, National Center for Multiple Sclerosis, Melsbroek, Belgium and Center for Neurosciences, Universitair Ziekenhuis Brussel en Vrije Universiteit Brussel, Belgium;3. Laboratory of Neurology, Born Bunge Institute, Translational Neurosciences, Faculty and Health Sciences, University of Antwerp and Division of Neurology, Antwerp University Hospital, Edegem, Belgium;4. Department of Scientific Coordination and Biostatistics, Antwerp University Hospital, Edegem, Belgium;1. Center for Cell and Gene Therapy, Texas Children''s Hospital, Baylor College of Medicine, Houston, Texas, USA;2. Texas Children''s Cancer Center, Texas Children''s Hospital, Baylor College of Medicine, Houston, Texas, USA;3. Interdepartmental Program in Translational Biology and Molecular Medicine, Baylor College of Medicine, Houston, Texas, USA;4. Department of Pediatrics, Baylor College of Medicine, Houston, Texas, USA;5. Department of Pathology and Immunology, Baylor College of Medicine, Houston, Texas, USA;1. Oncoematologia Pediatrica, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy;2. Dipartimento di Oncoematologia Pediatrica, IRCCS Ospedale Pediatrico Bambino Gesù, Roma, Italy;3. Servizio di Immunoematologia e Medicina Trasfusionale, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy;4. Struttura Complessa di Radioterapia Oncologica, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy;5. Department of Pediatric Science, Università degli Studi di Pavia, Italy |
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| Abstract: | Background aimsFanconi anemia is an autosomal recessive or X-linked genetic disorder characterized by bone marrow (BM) failure/aplasia. Failure of hematopoiesis results in depletion of the BM stem cell reservoir, which leads to severe anemia, neutropenia and thrombocytopenia, frequently requiring therapeutic interventions, including hematopoietic stem cell transplantation (HSCT). Successful BM transplantation (BMT) requires reconstitution of normal immunity.MethodsIn the present study, we performed a detailed analysis of the distribution of peripheral blood subsets of T, B and natural killer (NK) lymphocytes in 23 patients with Fanconi anemia before and after BMT on days +30, +60, +100, +180, +270 and +360. In parallel, we evaluated the effect of related versus unrelated donor marrow as well as the presence of graft-versus-host disease (GVHD).ResultsAfter transplantation, we found different kinetics of recovery for the distinct major subsets of lymphocytes. NK cells were the first to recover, followed by cytotoxic CD8+ T cells and B cells, and finally CD4+ helper T cells. Early lymphocyte recovery was at the expense of memory cells, potentially derived from the graft, whereas recent thymic emigrant (CD31+ CD45RA+) and naive CD4+ or CD8+ T cells rose only at 6 months after HSCT, in the presence of immunosuppressive GVHD prophylactic agents. Only slight differences were observed in the early recovery of cytotoxic CD8+ T cells among those cases receiving a graft from a related donor versus an unrelated donor. Patients with GVHD displayed a markedly delayed recovery of NK cells and B cells as well as of regulatory T cells and both early thymic emigrant and total CD4+ T cells.ConclusionsOur results support the utility of post-transplant monitoring of a peripheral blood lymphocyte subset for improved follow-up of patients with Fanconi anemia undergoing BMT. |
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| Keywords: | bone marrow Fanconi anemia immune system transplantation |
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