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获得性无巨核细胞性血小板减少症迅速进展为骨髓增生异常综合征:病例报告

Rapid progression of acquired amegakaryocytic thrombocytopenia to myelodysplastic syndrome: case report.

作者信息

Erkurt Mehmet Ali, Kaya Emin, Baran Murat, Yitmen Ece, Şenel Soner, Kuku İrfan, Aydoğdu İsmet

出版信息

Turk J Haematol. 2005 Dec 5;22(4):205-8.

PMID:27265013
Abstract

Acquired amegakaryocytic thrombocytopenia (AAT) is a rare disease characterized by severe thrombocytopenia due to selective reduction or absence of megakaryocytes in the bone marrow. More commonly, patients with AAT have additional hematologic abnormalities such as macrocytosis or dyserythropoiesis, abnormalities which may predict progression to aplastic anemia or myelodysplasia. A 52-year-old female was admitted to hospital with mucosal and gingival bleeding. Megakaryocytes were not seen on the bone marrow aspiration and biopsy. AAT was diagnosed. Although she was treated with immunosuppressive therapy including prednisolone and cyclosporine, her disease progressed to myelodysplastic syndrome. She died at the third month of diagnosis because of cerebral bleeding.

摘要

获得性无巨核细胞性血小板减少症(AAT)是一种罕见疾病,其特征是由于骨髓中巨核细胞选择性减少或缺失导致严重血小板减少。更常见的情况是,AAT患者还存在其他血液学异常,如大细胞性贫血或红细胞生成异常,这些异常可能预示着会进展为再生障碍性贫血或骨髓增生异常综合征。一名52岁女性因黏膜和牙龈出血入院。骨髓穿刺和活检未发现巨核细胞。诊断为AAT。尽管她接受了包括泼尼松龙和环孢素在内的免疫抑制治疗,但其病情仍进展为骨髓增生异常综合征。她在诊断后的第三个月因脑出血死亡。

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Acquired amegakaryocytic thrombocytopenia as a rare cause of thrombocytopenia during pregnancy.
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BMJ Case Rep. 2019 Jun 21;12(6):e230361. doi: 10.1136/bcr-2019-230361.
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[Acquired amegacaryocytic thrombocytopenic purpura hiding acute myeloid leukemia].[获得性巨核细胞性血小板减少性紫癜掩盖急性髓系白血病]
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Successful treatment of acquired amegakaryocytic thrombocytopenic purpura refractory to corticosteroids and intravenous immunoglobulin with antithymocyte globulin and cyclosporin.采用抗胸腺细胞球蛋白和环孢素成功治疗对皮质类固醇和静脉注射免疫球蛋白难治的获得性无巨核细胞性血小板减少性紫癜。
Int J Hematol. 2008 Sep;88(2):223-226. doi: 10.1007/s12185-008-0125-0. Epub 2008 Jul 15.