Aplastic Anemia and Other Bone Marrow Failure SyndromesNasrollah T. Shahidi During the past decade, there have been numerous direct and indirect scientific contributions to both the etiology and therapy of aplastic anemia and related bone marrow failure syndromes. Clinical observations, such as autologous bone marrow recovery after conditioning with immunosup pressive agents for bone marrow transplantation; failure to achieve en graftment in some identical twins without prior immunosuppressive ther apy; and hematologic response to immunosuppressive agents, have led to the concept of immune-mediated etiology of acquired aplastic anemia. Such a concept was further strengthened by laboratory findings, implicat ing the role of activated cytotoxic T lymphocytes and abnormal produc tion of inhibitory lymphokines. The immunologic mechanisms may also apply to the idiosyncratic bone marrow aplasias associated with drugs, toxic chemicals, and viruses. These agents may alter normal cellular recog nition sites by interacting with cellular components and result in loss of self tolerance. Immunologic mechanisms have long been advocated in many other organ failures, and the hemopoietic organ is no exception. It is of interest that parallel clinical and laboratory investigations in juvenile diabetes mellitus type I and in rodent models of this disease have yielded results compatible with the same pathogenic mechanisms. The infiltration of pancreatic islets by activated T lymphocytes, functional and morphological alterations of islet cells upon incubation with lymphokines such as gamma interferon and tumor necrosis factor, and clinical response to cyclosporine are a few examples. |
Contents
Contents | 1 |
Classification | 25 |
Constitutional Aplastic Anemia | 38 |
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Aplastic Anemia and Other Bone Marrow Failure Syndromes Nasrollah T. Shahidi No preview available - 2011 |
Aplastic Anemia and Other Bone Marrow Failure Syndromes Nasrollah T. Shahidi No preview available - 2011 |
Common terms and phrases
abnormalities accessory cells activity acute leukemia agents androgens antibodies antilymphocyte globulin antithymocyte globulin aplasia associated autologous BFU-E Blackfan blood cell bone marrow cells bone marrow failure bone marrow transplantation cellular CFU-E CFU-GM chronic Clin Invest clinical colony formation congenital hypoplastic corticosteroids culture cyclophosphamide cyclosporine cytogenetic diagnosis differentiation donor drugs effect Engl erythroid erythroid colony erythropoiesis etiocholanolone Exp Hematol Fanconi Fanconi anemia Gluckman GM-CSF graft-versus-host disease granulocyte growth factors GVHD hematologic hematopoietic hemopoietic human bone marrow hypoplastic anemia immunosuppressive infection inhibition inhibitory interferon interleukin lymphocytes M.D. Department macrophages monocytes myelodysplastic syndromes myeloid neutrophil pancytopenia patients with aplastic patients with severe peripheral blood platelet PRCA prednisone preleukemic syndrome progenitor cells proliferation pure red cell red cell aplasia remission reported response severe aplastic anemia stem cells steroids stimulating Storb studies suppression survival Tavassoli therapy tion toxic transfusion Treatment of aplastic viral vitro y-IFN Zanjani