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一名遗传性球形红细胞增多症患者因胸内髓外造血导致大量血胸。

Massive hemothorax due to intrathoracic extramedullary hematopoiesis in a patient with hereditary spherocytosis.

作者信息

Xiros N, Economopoulos T, Papageorgiou E, Mantzios G, Raptis S

机构信息

Second Department of Internal Medicine-Propaedeutic, Evangelismos Hospital, University of Athens, Greece.

出版信息

Ann Hematol. 2001 Jan;80(1):38-40. doi: 10.1007/s002770000222.

Abstract

Extramedullary hematopoiesis (EMH) is a rare disorder, characterized by the appearance of hematopoietic elements outside of the bone marrow, which occurs in patients with chronic myeloproliferative disorders or congenital hemolytic anemias. We report on a 64-year-old man with hereditary spherocytosis, who presented with anemia, jaundice, intrathoracic EMH, and massive hemothorax. The diagnosis of EMH was established after computer tomography (CT)-guided punctuation of the paravertebral mass. The patient underwent splenectomy and thoracic drainage. After 1 year, the patient is in good health, with normal hemoglobin values, and hemothorax has not recurred.

摘要

髓外造血(EMH)是一种罕见的疾病,其特征是骨髓外出现造血成分,发生于慢性骨髓增殖性疾病或先天性溶血性贫血患者。我们报告一例64岁患有遗传性球形红细胞增多症的男性患者,其表现为贫血、黄疸、胸内髓外造血和大量血胸。经计算机断层扫描(CT)引导下对椎旁肿块进行穿刺后确诊为髓外造血。该患者接受了脾切除术和胸腔引流。1年后,患者身体健康,血红蛋白值正常,血胸未复发。

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