Extramedullary hematopoiesis causing paraparesis in congenital cyanotic heart disease
Extramedullary hematopoiesis causing paraparesis in congenital cyanotic heart disease
复制标题
先天性紫绀型心脏病髓外造血导致截瘫
DOI：
10.1212/wnl.51.2.636
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发表时间：
1998
期刊：
影响因子：
9.9
通讯作者：
M. Hlavin
中科院分区：
文献类型：
--
作者：
C. Taylor;Frederick A. Maynard;J. Liebman;M. Hlavin
髓外造血（EH）是一种组织缺氧的代偿机制，通常由慢性贫血引起，通常发生在脾脏、淋巴结或肝脏。尽管已被认识到，但椎管内EH引起的轻瘫并不常见，报告了80多例病例，最常见于β地中海贫血或骨髓纤维化患者。其他诱发病症包括红细胞增多症、佩吉特病、含铁血黄素沉着症、白血病、霍奇金病或其他贫血状态（例如，铁粒幼细胞、镰状细胞、溶血性、丙酮酸激酶缺乏）。脑实质、脑膜和颅骨板障是罕见的部位。既往曾有一例因紫绀型心脏病导致EH累及脊髓的报道。1本文介绍的是一例先天性心脏病伴EH脊髓压迫的患者，经积极治疗后恢复了功能。
病例报告。这位26岁的男性出生时患有右心室发育不良和三尖瓣闭锁。童年中风导致智力迟钝和右侧轻偏瘫。在22岁之前，他可以用最小的权利跑步...
Extramedullary hematopoiesis (EH) is a compensatory mechanism for tissue hypoxia, usually due to chronic anemia, and typically occurs in the spleen, lymph nodes, or liver. Paraparesis from EH in the spinal canal is uncommon, although recognized, with over 80 cases reported, most frequently in patients with β-thalassemia or myelofibrosis. Other predisposing conditions include polycythemia, Paget's disease, hemosiderosis, leukemia, Hodgkin's disease, or other anemic states (e.g., sideroblastic, sickle cell, hemolytic, pyruvate kinase deficiency). The cerebral parenchyma, meninges, and skull diploe are rare sites. Spinal involvement by EH due to cyanotic heart disease has been reported once previously.1 Presented here is a patient with congenital heart disease and spinal cord compression by EH who recovered function with aggressive treatment.
Case report. This 26-year-old man was born with a hypoplastic right ventricle and tricuspid atresia. A childhood stroke produced mental retardation and right hemiparesis. Before age 22 years, he could run with minimal right …