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与明显的爱泼斯坦-巴尔病毒感染相关的无巨核细胞性血小板减少症及随后的再生障碍性贫血

Amegakaryocytic Thrombocytopenia and Subsequent Aplastic Anemia Associated with Apparent Epstein-Barr Virus Infection.

作者信息

Levy Ilana, Laor Ruth, Jiries Nizar, Bejar Jacob, Polliack Aaron, Tadmor Tamar

机构信息

Internal Medicine B Department, Bnai Zion Medical Center, Haifa, Israel.

出版信息

Acta Haematol. 2018;139(1):7-11. doi: 10.1159/000484595. Epub 2018 Jan 5.

Abstract

Acquired amegakaryocytic thrombocytopenia (AAT), a rare entity characterized by severe thrombocytopenia and the absence of megakaryocytes in the bone marrow, may mimic or precede the diagnosis of aplastic anemia (AA). Here, we describe a patient who presented with apparent Epstein-Barr virus (EBV)-associated immune thrombocytopenia resistant to several lines of therapies, which was in fact a form of AAT with some features of AA. He eventually responded to therapy with eltrombopag, cyclosporine A (CSA), and antithymocyte globulin (ATG) and recovered completely. EBV infection is known to cause a variety of benign and malignant hematologic disorders, including bone marrow failure. However, to the best of our knowledge, this is the first case report of EBV-associated AAT. Treatment options for AAT are still not well defined, and even response to eltrombopag together with CSA and ATG does not always imply successful therapy. The natural history of EBV infection may well be sufficient to explain unexpected eventual recovery.

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,其特征为严重血小板减少且骨髓中无巨核细胞,可能会模拟再生障碍性贫血(AA)的诊断或在其诊断之前出现。在此,我们描述一名患者,其表现为明显的对多种治疗耐药的爱泼斯坦-巴尔病毒(EBV)相关免疫性血小板减少症,而实际上这是一种具有AA某些特征的AAT形式。他最终对艾曲泊帕、环孢素A(CSA)和抗胸腺细胞球蛋白(ATG)治疗产生反应并完全康复。已知EBV感染会导致多种良性和恶性血液系统疾病,包括骨髓衰竭。然而,据我们所知,这是首例EBV相关AAT的病例报告。AAT的治疗选择仍未明确界定,即使对艾曲泊帕联合CSA和ATG有反应也并不总是意味着治疗成功。EBV感染的自然病程很可能足以解释意外的最终康复情况。

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