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真性红细胞增多症患者出现心脏压塞的髓外造血。

Extramedullary haematopoiesis presenting with cardiac tamponade in a patient with polycythaemia vera.

作者信息

Haroun Faysal, Elkis Viktoria, Chen Anne, Lee Elsie

机构信息

Division of Hematology and Oncology, Department of Medicine, George Washington University School of Medicine and Health Sciences, Washington, USA.

Department of Medicine, George Washington University School of Medicine and Health Sciences, Washington, USA.

出版信息

BMJ Case Rep. 2017 Aug 10;2017:bcr-2017-221316. doi: 10.1136/bcr-2017-221316.

DOI:10.1136/bcr-2017-221316
PMID:28798246
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC5624000/
Abstract

A 71-year-old man with a history of polycythaemia vera, diagnosed 4 years ago, presented to the emergency room with shortness of breath. A bedside echocardiogram revealed a large pericardial effusion with features concerning for pericardial tamponade. A left anterior thoracotomy and a pericardial window were emergently performed in the operating room and relieved the patient's symptoms. Histology evaluation of the pericardial fragments and pericardial fluid revealed the presence of trilineage haematopoietic elements without any increase in the blasts. A bone marrow core biopsy revealed an increase in reticulin fibre and increase in the number of blasts of 5%-10%, whereas peripheral blood testing was positive for JAK2 V617F mutation. This case report reviews the literature for cases of extramedullary haematopoiesis associated with myeloproliferative neoplasms.

摘要

一名71岁男性,4年前被诊断为真性红细胞增多症,因呼吸急促入住急诊室。床旁超声心动图显示大量心包积液,具有心包填塞的特征。在手术室紧急进行了左前开胸手术和心包开窗术,缓解了患者的症状。心包碎片和心包液的组织学评估显示存在三系造血成分,原始细胞无增加。骨髓芯活检显示网状纤维增加,原始细胞数量增加5%-10%,而外周血检测JAK2 V617F突变呈阳性。本病例报告回顾了与骨髓增殖性肿瘤相关的髓外造血病例的文献。

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引用本文的文献

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本文引用的文献

1
Myeloproliferative Neoplasms.骨髓增殖性肿瘤
N Engl J Med. 2017 Jun 1;376(22):2168-2181. doi: 10.1056/NEJMra1406186.
2
Prognostic impact of bone marrow fibrosis in polycythemia vera: validation of the IWG-MRT study and additional observations.真性红细胞增多症中骨髓纤维化的预后影响:IWG-MRT研究的验证及其他观察结果
Blood Cancer J. 2017 Mar 10;7(3):e538. doi: 10.1038/bcj.2017.17.
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Update from the latest WHO classification of MPNs: a user's manual.世界卫生组织髓系增殖性肿瘤最新分类更新:用户指南。
Hematology Am Soc Hematol Educ Program. 2016 Dec 2;2016(1):534-542. doi: 10.1182/asheducation-2016.1.534.
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Case report and literature review: cardiac tamponade as a complication of pericardial extramedullary hematopoiesis.病例报告与文献综述:心包髓外造血并发心脏压塞
Cardiovasc Pathol. 2016 Sep-Oct;25(5):371-4. doi: 10.1016/j.carpath.2016.05.007. Epub 2016 May 30.
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The 2016 revision of the World Health Organization classification of lymphoid neoplasms.《世界卫生组织淋巴组织肿瘤分类(2016年修订版)》
Blood. 2016 May 19;127(20):2375-90. doi: 10.1182/blood-2016-01-643569. Epub 2016 Mar 15.
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A Rare Presentation of Extramedullary Hematopoiesis in Post-polycythemic Myelofibrosis.真性红细胞增多症后骨髓纤维化中髓外造血的罕见表现
Indian J Hematol Blood Transfus. 2014 Sep;30(Suppl 1):12-6. doi: 10.1007/s12288-012-0218-z. Epub 2012 Dec 7.
7
Long-term survival and blast transformation in molecularly annotated essential thrombocythemia, polycythemia vera, and myelofibrosis.分子注释的原发性血小板增多症、真性红细胞增多症和骨髓纤维化的长期生存及原始细胞转化
Blood. 2014 Oct 16;124(16):2507-13; quiz 2615. doi: 10.1182/blood-2014-05-579136. Epub 2014 Jul 18.
8
Survival and prognosis among 1545 patients with contemporary polycythemia vera: an international study.1545 例当代真性红细胞增多症患者的生存和预后:一项国际研究。
Leukemia. 2013 Sep;27(9):1874-81. doi: 10.1038/leu.2013.163. Epub 2013 Jun 6.
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A rare cause of cardiac tamponade.
Acta Cardiol. 2012 Dec;67(6):719-21. doi: 10.1080/ac.67.6.2184676.
10
Extramedullary hematopoiesis: a new look at the underlying stem cell niche, theories of development, and occurrence in animals.髓外造血:对基础造血干细胞龛、发育理论以及动物发生的新认识。
Vet Pathol. 2012 May;49(3):508-23. doi: 10.1177/0300985811432344. Epub 2012 Jan 18.