Jeffrey B Booker, Kenneth M Zabel, Jake Soens, Abu Baker Sheikh
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引用次数: 0
Abstract
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.
巨核细胞性血小板减少症是一种罕见的血液病,其特点是由于巨核细胞几乎缺失,血小板生成严重减少,导致严重的血小板减少症。本报告阐明了在处理这种罕见血液病和严重心血管疾病时所遇到的诊断和治疗挑战。我们详细介绍了一名患者的病例,该患者因胸痛就诊,被诊断为非 ST 段抬高型心肌梗死(NSTEMI)。随后的检查发现他患有严重的血小板减少症和潜在的三血管疾病,这使得立即进行外科干预变得复杂。最初的治疗策略旨在治疗假定的免疫性血小板减少症,但效果不佳。经过骨髓活检,最终确诊为巨核细胞性血小板减少症。尽管调整了治疗方案,包括使用促血小板生成素激动剂和免疫抑制剂,血小板数量还是有所改善,但仍未达到冠状动脉旁路移植术的安全水平。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。该病例还说明了在治疗复杂病例时跨学科合作的重要性:巨核细胞血小板减少症是一种罕见的血小板减少症,可与心脏疾病并发。巨核细胞血小板减少症的诊断可能很困难,因为它可能被误认为是免疫介导的血小板减少症,但由于治疗方法不同,区分巨核细胞血小板减少症至关重要。
期刊介绍:
The European Journal of Case Reports in Internal Medicine is an official journal of the European Federation of Internal Medicine (EFIM), representing 35 national societies from 33 European countries. The Journal''s mission is to promote the best medical practice and innovation in the field of acute and general medicine. It also provides a forum for internal medicine doctors where they can share new approaches with the aim of improving diagnostic and clinical skills in this field. EJCRIM welcomes high-quality case reports describing unusual or complex cases that an internist may encounter in everyday practice. The cases should either demonstrate the appropriateness of a diagnostic/therapeutic approach, describe a new procedure or maneuver, or show unusual manifestations of a disease or unexpected reactions. The Journal only accepts and publishes those case reports whose learning points provide new insight and/or contribute to advancing medical knowledge both in terms of diagnostics and therapeutic approaches. Case reports of medical errors, therefore, are also welcome as long as they provide innovative measures on how to prevent them in the current practice (Instructive Errors). The Journal may also consider brief and reasoned reports on issues relevant to the practice of Internal Medicine, as well as Abstracts submitted to the scientific meetings of acknowledged medical societies.