Liang Hui, Xu Ziqi, Zheng Zhijun, Lou Haiyan, Yue Wei
Department of Neurology, The First Affiliated Hospital, School of Medicine, Zhejiang University, Hangzhou, China.
Department of Neurology, Hengdian hospital of Zhejiang Province, Zhejiang, China.
Orphanet J Rare Dis. 2015 Apr 22;10:47. doi: 10.1186/s13023-015-0268-z.
Reversible cerebral vasoconstriction syndrome (RCVS) is an infrequent disease characterized by severe headaches with or without focal neurological deficits or seizures and a reversible vasoconstriction of cerebral arteries. The Orpha number for RCVS is ORPHA284388. However, RCVS triggered by blood transfusion is rare. Here we provided the clinical, neuroimaging and outcome data of patients diagnosed with RCVS resulting from red blood cells transfusion.
We retrospectively identified 7 patients presenting with RCVS after red blood cells transfusion from January 2010 to May 2014. The information on clinical features, neuroimaging and outcome were collected and analyzed.
All 7 patients were Chinese women, with a mean age of 42 years (38-46). All the patients had severe anemia (Hb level < 6 g/dl) caused by primary menorrhagia due to uterine myoma (n = 5) or end-stage renal disease (n = 2) and severe anemia persisted for a average period of 4 months (2-6). Each patient received packed red blood cells transfusion (average: 1580 ml) over a period of 2-5 days. Blood transfusion increased the hemoglobin level by at least 4.5 g/dL from baseline. The neurological symptoms appeared a mean of 6.3 days (2-13) after the last blood transfusion. Headache was the most frequent symptom and seizure, transient or persistent neurological disorders were observed. Neuroimaging showed cortical subarachnoid hemorrhage (n = 2), focal intracerebral hemorrhage (n = 2), localized brain edema (n = 3), cerebral infarction (n = 1), and posterior reversible encephalopathy syndrome (n = 2). Cerebral vasoconstrictions were demonstrated by magnetic resonance angiography or cerebral angiography. Arterial constriction reversed in all patients within 1 to 3 months of follow-up after disease onset and no relapse was observed up to a mean of 17.1 ± 4.8 months of follow-up.
RCVS is a rare complication as a result of blood transfusion in patients with chronic severe anemia and should be considered in patients who show severe headache or neurologic deficits after transfusion.
可逆性脑血管收缩综合征（RCVS）是一种罕见疾病，其特征为伴有或不伴有局灶性神经功能缺损或癫痫发作的严重头痛以及脑动脉的可逆性血管收缩。RCVS的孤儿病编号为ORPHA284388。然而，由输血引发的RCVS很罕见。在此，我们提供了被诊断为由红细胞输血导致的RCVS患者的临床、神经影像学及转归数据。
我们回顾性确定了2010年1月至2014年5月间7例红细胞输血后出现RCVS的患者。收集并分析了临床特征、神经影像学及转归方面的信息。
所有7例患者均为中国女性，平均年龄42岁（38 - 46岁）。所有患者均因子宫肌瘤（n = 5）或终末期肾病（n = 2）导致的原发性月经过多而患有严重贫血（血红蛋白水平<6 g/dl），且严重贫血平均持续4个月（2 - 6个月）。每位患者在2 - 5天内接受了浓缩红细胞输血（平均：1580 ml）。输血使血红蛋白水平较基线至少升高4.5 g/dL。神经症状在最后一次输血后平均6.3天（2 - 13天）出现。头痛是最常见的症状，还观察到癫痫发作、短暂或持续性神经功能障碍。神经影像学显示皮质下蛛网膜下腔出血（n = 2）、局灶性脑出血（n = 2）、局限性脑水肿（n = 3）、脑梗死（n = 1）以及后部可逆性脑病综合征（n = 2）。磁共振血管造影或脑血管造影证实了脑血管收缩。发病后随访1至3个月内所有患者的动脉收缩均逆转，平均随访17.1±4.8个月未见复发。
RCVS是慢性严重贫血患者输血导致的罕见并发症，对于输血后出现严重头痛或神经功能缺损的患者应考虑此病。
