Pathirage Loku Pathirage Manoji Muditha Kumari, Wijeweera Indunil, Jayasinghe Chandrika, Jayabahu Saumya, Wickramasinghe Hasith Ravinda
Department of Medicine, Faculty of Medicine, University of Peradeniya, Peradeniya, Sri Lanka.
Neurology Unit, General Hospital - Kandy, Kandy, Sri Lanka.
BMC Res Notes. 2016 Feb 25;9:123. doi: 10.1186/s13104-016-1918-5.
Extramedullary haemopoiesis is a common compensatory phenomenon in most haemolytic anaemias. However, spinal cord compression due to extramedullary spinal epidural haemopoiesis is an extremely rare complication of thalassemia. In such situation patients present with paraplegia with a sensory level. Usual treatment options are surgery and/or radiotherapy.
Here we report a 27 year old Sri Lankan Muslim male with haemoglobin E-Beta thalassaemia presented with episodic spastic paraparesis when he was anaemic which was dramatically responded to blood transfusion therapy.
Most of the reported cases with paraplegia have been treated with surgery with or without radiation therapy or radiation therapy alone. Our patient makes dramatic recovery after blood transfusion in each presentation.
髓外造血是大多数溶血性贫血中常见的代偿现象。然而,脊髓硬膜外髓外造血导致的脊髓压迫是地中海贫血极为罕见的并发症。在这种情况下,患者表现为伴有感觉平面的截瘫。常用的治疗选择是手术和/或放疗。
在此,我们报告一名27岁的斯里兰卡穆斯林男性,患有血红蛋白E-β地中海贫血,在贫血时出现发作性痉挛性截瘫,输血治疗后有显著反应。
大多数报道的截瘫病例已接受手术治疗,联合或不联合放射治疗,或仅接受放射治疗。我们的患者每次发作后输血治疗均有显著恢复。