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获得性无巨核细胞性血小板减少症合并严重冠状动脉疾病的管理

Management of Acquired Amegakaryocytic Thrombocytopenia and Severe Coronary Artery Disease.

作者信息

Booker Jeffrey B, Zabel Kenneth M, Soens Jake, Sheikh Abu Baker

机构信息

School of Medicine, University of New Mexico, Albuquerque, USA.

Department of Internal Medicine, University of New Mexico, Albuquerque, USA.

出版信息

Eur J Case Rep Intern Med. 2024 Sep 19;11(10):004736. doi: 10.12890/2024_004736. eCollection 2024.

Abstract

UNLABELLED

Amegakaryocytic thrombocytopenia is a rare haematologic disorder characterised by a profound reduction in platelet production due to the near absence of megakaryocytes, leading to severe thrombocytopenia. This report elucidates the diagnostic and therapeutic challenges encountered in managing such rare haematological conditions alongside significant cardiovascular disease. We detail the case of a patient who presented with chest pain and was diagnosed with non-ST elevation myocardial infarction (NSTEMI). Subsequent investigations revealed severe thrombocytopenia and underlying triple vessel disease, complicating immediate surgical intervention. Initial management strategies aimed at treating presumed immune thrombocytopenia proved ineffective. A definitive diagnosis of amegakaryocytic thrombocytopenia was established following a bone marrow biopsy. Despite treatment adjustments, including the administration of thrombopoietin agonists and immunosuppression, platelet counts improved but did not reach levels safe for coronary artery bypass grafting. This case underscores the importance of diagnosis and treatment of systemic disorders prior treatment of cardiac disease. It also demonstrates the importance of interdisciplinary cooperation in the treatment of a complex patient case.

LEARNING POINTS

Amegakaryocytic thrombocytopenia is a rare cause of thrombocytopenia that can co-occur with cardiac conditions.The diagnosis of amegakaryocytic thrombocytopenia can be difficult as it can be mistaken for immune mediated thrombocytopenia, but distinction is critical as treatments differ.Modern patient care frequently requires collaboration between different subspecialities.

摘要

未标注

无巨核细胞性血小板减少症是一种罕见的血液系统疾病,其特征是由于几乎没有巨核细胞导致血小板生成显著减少,进而导致严重的血小板减少症。本报告阐明了在管理此类罕见血液疾病以及严重心血管疾病时遇到的诊断和治疗挑战。我们详细介绍了一名出现胸痛并被诊断为非ST段抬高型心肌梗死(NSTEMI)的患者的病例。随后的检查发现严重血小板减少症和潜在的三支血管病变,使立即进行手术干预变得复杂。旨在治疗疑似免疫性血小板减少症的初始管理策略被证明无效。骨髓活检后确诊为无巨核细胞性血小板减少症。尽管调整了治疗方案,包括使用血小板生成素激动剂和免疫抑制,但血小板计数有所改善,但未达到冠状动脉旁路移植术安全的水平。该病例强调了在治疗心脏病之前诊断和治疗全身性疾病的重要性。它还展示了跨学科合作在治疗复杂患者病例中的重要性。

学习要点

无巨核细胞性血小板减少症是血小板减少症的罕见原因,可与心脏疾病同时发生。无巨核细胞性血小板减少症的诊断可能困难,因为它可能被误诊为免疫介导的血小板减少症,但区分至关重要,因为治疗方法不同。现代患者护理经常需要不同亚专业之间的合作。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/26fe/11451836/83e66687e996/4736_Fig1.jpg

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