Haroun Faysal, Elkis Viktoria, Chen Anne, Lee Elsie
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.
BMJ Case Rep. 2017 Aug 10;2017:bcr-2017-221316. doi: 10.1136/bcr-2017-221316.
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.
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。