原发性心脏淋巴瘤表现为血小板减少、右心衰和心源性休克。

IF 0.7 Q4 HEMATOLOGY
Samantha Kurniawan, Gita Mathur, Yvonne Bogun, Giselle Kidson-Gerber
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引用次数: 0

摘要

原发性心脏淋巴瘤(PCL)是一种罕见的、潜在致命的非霍奇金淋巴瘤亚型。血小板减少症也很少与其他原发性心脏肿瘤相关,如果存在,可能会增加已经危及生命的诊断的大量发病率。我们报告一例罕见的70岁男性患者,其表现为血小板减少(91 × 109/L)和进行性右心衰。经胸超声心动图示8 × 4 cm大右心房肿块伴严重三尖瓣梗阻,经心肌内膜活检及免疫组化证实为PCL。他恶化为心房颤动诱发的心源性休克,伴缺血性肝炎时血小板减少加重(18 × 109/L)。患者在组织诊断前开始使用大剂量类固醇后病情稳定,化疗后血小板计数恢复正常。本病例证明了将PCL作为诊断的重要性,以及先发制人地使用大剂量类固醇来改善PCL合并心源性休克的预后。这个病例也阐明了PCL和血小板减少症之间潜在的病理生理联系。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

Primary Cardiac Lymphoma Presenting with Thrombocytopenia, Right Heart Failure, and Cardiogenic Shock.

Primary Cardiac Lymphoma Presenting with Thrombocytopenia, Right Heart Failure, and Cardiogenic Shock.

Primary Cardiac Lymphoma Presenting with Thrombocytopenia, Right Heart Failure, and Cardiogenic Shock.

Primary Cardiac Lymphoma Presenting with Thrombocytopenia, Right Heart Failure, and Cardiogenic Shock.

Primary cardiac lymphoma (PCL) is a rare, potentially fatal subtype of non-Hodgkin's lymphoma. Thrombocytopenia has also infrequently been reported in association with other primary cardiac tumours and can add substantial morbidity to an already life-threatening diagnosis if present. We report a rare case of a 70-year-old man who presented with thrombocytopenia (91 × 109/L) and progressive right heart failure. Transthoracic echocardiogram revealed a large 8 × 4 cm right atrial mass with severe tricuspid obstruction, confirmed as PCL on subsequent endomyocardial biopsy and immunohistochemistry. He deteriorated into cardiogenic shock precipitated by atrial fibrillation, with worsening thrombocytopenia (18 × 109/L) in the setting of ischaemic hepatitis. The patient stabilised with initiation of high dose steroids prior to tissue diagnosis and platelet counts normalised following chemotherapy. This case demonstrates the importance of considering PCL as a diagnosis and preemptive initiation of high dose steroids to improve outcomes in PCL associated with cardiogenic shock. This case also elucidates a potential pathophysiological association between PCL and thrombocytopenia.

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