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Abstract

第2巻 2006年6月 第3号

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■綜説

再生不良性貧血―病態生理と治療―
田近 賢二
日本医科大学内科学講座血液内科

Aplastic Anemia; Current Findings of its Pathophysiology and Treatment
Kenji Tajika
Department of Internal Medicine, Division of Hematology

Results of T-cell receptor β-chain repertoire analysis, gene expression profiling studies of CD34-positive cells from patients with aplastic anemia, and other cytokine data have shown that the major pathogenic mechanism of acquired aplastic anemia is stem cell injury by morbid T-cells. Several proteins, such as kinectin and diazepam-binding inhibitor-related protein 1, have been found to be related to the onset as self-antigens. With these findings and additional new clues the pathogenesis of aplastic anemia will soon be clarified. The current strategies to treat severe aplastic anemia are stem cell transplantation and immunosuppressive therapy with anti-thymocyte globulin and cyclosporine. These methods have both benefits and some problems, so it is sometimes not easy to determine which therapy should be applied to patients. Some data suggest that the presence of paroxysmal nocturnal hemoglobinuria (PNH) clones in patient's peripheral blood heralds a good response to immunosuppressive agents. PHN clones may be a useful tool for selecting therapy. For stem cell transplantation, an HLA-identical sibling is the best donor candidate, but such a donor is available for only 30% of patients. Recently, HLA-mismatched related or HLA-matched unrelated bone marrow cells and HLA-mismatched umbilical cord blood cells have been used as alternative stem cell sources if an HLA-identical sibling donor is not available. However, greater efforts must be made to reduce the mortality and morbidity rates of stem cell transplantation. In this paper recent progress in the basic research and treatment of aplastic anemia are reviewed.

日医大医会誌 2006; 2(3), 138-144

Key words
aplastic anemia, immune disorder, immunosuppressive therapy, stem cell transplantation

Correspondence to
Kenji Tajika, Department of Internal Medicine, Division of Hematology, Nippon Medical School, 1-1-5 Sendagi, Bunkyo-ku, Tokyo 113-8603, Japan
E-mail:tajika@nms.ac.jp

受付:2005年3月31日 受理:2005年5月16日

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