Immune-induced cytopenia: bone marrow failure syndrome.
Myelodysplastic syndromes, a heterogeneous group of clonal stem cell disorders, are characterized by ineffective and dysplastic hematopoiesis and increased risk of leukemic transformation. The clonal disorder may result in cumulative cytogenetic abnormalities in a multistep process. Intrinsic and extrinsic factors contributing to ineffective hematopoiesis include genetic predisposition, environmental effects, and iatrogenic causes. The common and often overlapping features of myelodysplastic syndrome, aplastic anemia, T-cell large granular lymphocyte lymphoproliferative disorder, and paroxysmal nocturnal hemoglobinuria suggest a shared pathophysiologic mechanism of marrow failure. Recent studies suggest that dominant clonal T cells, which may be driven to expand by hematopoietic antigenic stimuli, result in the T-cell-mediated inhibition of hematopoietic progenitors, thus contributing to cytopenia.
Kondo
Yukio
Y
Department of Blood and Marrow Transplantation, University of Texas MD Anderson Cancer Center, 7455 Fannin SCRB-Unit 900, PO Box 301402, Houston, TX 77230, USA.
Molldrem
Jeffrey J
JJ
D001855:Bone Marrow Diseases / Q000188:drug therapy / Q000276:immunology / Q000473:pathology; D006410:Hematopoiesis / Q000276:immunology; D006801:Humans; D007111:Immunity, Cellular; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D009190:Myelodysplastic Syndromes / Q000188:drug therapy / Q000276:immunology / Q000473:pathology*; D010198:Pancytopenia / Q000188:drug therapy / Q000276:immunology*; D013601:T-Lymphocytes / Q000276:immunology / Q000473:pathology*
D007166:Immunosuppressive Agents