获得性巨核细胞血小板减少症误诊为免疫性血小板减少症：病例报告。
Acquired Amegakaryocytic Thrombocytopenia Misdiagnosed as Immune Thrombocytopenia: A Case Report.
机构信息
John L McClellan Memorial Veterans Hospital, Little Rock, AR.
University of Arkansas for Medical Sciences, Little Rock, AR.
出版信息
Perm J. 2020 Dec;24:1-3. doi: 10.7812/TPP/19.203.
INTRODUCTION
Acquired amegakaryocytic thrombocytopenia (AATP) is a rare bleeding disorder that causes severe thrombocytopenia with preserved hematopoiesis of other cell lineages. Many cases are misdiagnosed and treated as immune thrombocytopenia.
CASE PRESENTATION
We report a case of AATP, in a 50-year-old man, that was treated as immune thrombocytopenia for years with no clinical response. The disorder later was diagnosed as AATP after bone marrow biopsy and was successfully treated with cyclosporine.
DISCUSSION
The exact mechanism of AATP remains unclear; it is suspected to be an immune-mediated process. Patients with AATP present with severe bleeding and thrombocytopenia, which is usually unresponsive to high-dose corticosteroids. There are no standard treatment guidelines for AATP. Cyclosporine and antithymocyte globulin are found to be effective in some cases. The prompt diagnosis of AATP is vital because it carries high mortality because of excessive bleeding, and it can progress into aplastic anemia or myelodysplastic syndrome.
简介
获得性巨核细胞减少性血小板减少症（AATP）是一种罕见的出血性疾病，可导致严重的血小板减少症，而其他细胞谱系的造血功能得以保留。许多病例被误诊并被误诊为免疫性血小板减少症。
病例介绍
我们报告了一例 AATP 病例，患者为 50 岁男性，多年来一直被误诊为免疫性血小板减少症，且无临床反应。后来，骨髓活检诊断为 AATP，并成功接受环孢素治疗。
讨论
AATP 的确切机制仍不清楚；据推测它是一种免疫介导的过程。AATP 患者表现为严重出血和血小板减少症，通常对大剂量皮质类固醇反应不佳。目前尚无 AATP 的标准治疗指南。环孢素和抗胸腺细胞球蛋白在某些情况下被发现有效。及时诊断 AATP 至关重要，因为大量出血会导致其死亡率较高，且它可能进展为再生障碍性贫血或骨髓增生异常综合征。
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