Suárez A Diana Vanessa, Del Rosario Alvarez V María, Rojas H Juan Pablo
Rev Chil Pediatr. 2014 Apr;85(2):203-6. doi: 10.4067/S0370-41062014000200010.
Extramedullary hematopoiesis (EMH) is defined as the formation and development of erythrocytic, granulocytic and megakaryocytic cell lines outside the bone marrow; it is a rare occurrence, which is limited to case reports.
To describe a case of renal EMH secondary to chronic hypoxia.
The patient is a 16 year old teenager who developed chronic hypoxia, central cyanosis, clubbing and lives more than 2,500 m above sea level. Chronic hypoxia is probably secondary to pulmonary hypertension, neither detected nor previously treated. The Consultation was due to a mass in the left upper quadrant for the last three months. An Echocardiogram showed pulmonary hypertension that could not be assessed by hemodynamic parameters. A computed tomography showed bilateral left renal pelvic masses. The hemogram showed polycythemia, so bone marrow aspiration was performed, which rule out malignant pathology. A biopsy of the renal mass reported EMH with three hematopoietic cell lines.
This EMH case is a response to chronic hypoxia rather than to a bone marrow lesion as it has usually been described.
髓外造血(EMH)定义为骨髓外红细胞、粒细胞和巨核细胞系的形成与发育;这是一种罕见情况,仅见于病例报告。
描述一例继发于慢性缺氧的肾髓外造血病例。
患者为一名16岁青少年,患有慢性缺氧、中心性发绀、杵状指,居住在海拔2500米以上地区。慢性缺氧可能继发于肺动脉高压,此前未检测到且未接受过治疗。此次会诊是因为过去三个月左上腹出现肿块。超声心动图显示肺动脉高压,但无法通过血流动力学参数评估。计算机断层扫描显示双侧左肾盂肿块。血常规显示红细胞增多,因此进行了骨髓穿刺,排除了恶性病变。肾肿块活检报告为具有三种造血细胞系的髓外造血。
该髓外造血病例是对慢性缺氧的反应,而非如通常所描述的对骨髓病变的反应。