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一名中间型地中海贫血女性患者,持续性咳嗽作为胸腔内髓外造血(EMH)的非典型临床表现。

A persistent cough as atypical clinical presentation of intrathoracic extramedullary hematopoiesis (EMH) in a female with thalassemia intermedia.

作者信息

Abdulla Mohammad A J, Yassin Mohamed A, Abdelrazek Mohamed, Mudawi Deena, Ibrahim Firyal, Soliman Dina S, ElOmri Halima, Nashwan Abdulqadir J, Fernyhough Liam Joseph, De Sanctis Vincenzo, Soliman Ashraf T

机构信息

Department of Medical Oncology, Hematology Section, National Center for Cancer Care and Research, Hamad Medical Corporation, Doha, Qatar.

出版信息

Acta Biomed. 2018 Feb 16;89(2-S):41-46. doi: 10.23750/abm.v89i2-S.7086.

Abstract

Extramedullary hematopoiesis (EMH) is a rare disorder, defined as the appearance of hematopoietic elements outside the bone marrow or peripheral blood. The most common sites of EMH are liver and spleen, but it has been documented in other organs such as the mediastinum, lymph nodes, breast, and central nervous system. EMH occurs as a compensatory mechanism for bone marrow dysfunction in severe thalassemia. We report a case of EMH presenting as a posterior mediastinal mass in a 34-year-old woman with thalassemia intermedia with chronic cough and shortness of breath on exertion. The diagnosis of EMH was confirmed by a CT-guided fine needle biopsy. All symptoms disappeared after surgical removal of the mass.

摘要

髓外造血(EMH)是一种罕见的病症,定义为骨髓或外周血以外出现造血成分。EMH最常见的部位是肝脏和脾脏,但在纵隔、淋巴结、乳腺和中枢神经系统等其他器官中也有记载。EMH作为重度地中海贫血骨髓功能障碍的一种代偿机制而发生。我们报告一例34岁中间型地中海贫血女性患者,其EMH表现为后纵隔肿块,伴有慢性咳嗽和劳力性气短。通过CT引导下细针穿刺活检确诊为EMH。手术切除肿块后所有症状均消失。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/33b6/6179037/2f72a9f6d817/ACTA-89-41-g001.jpg

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