Horn M, Steiner T, Günther T, Mendoza G, von Kummer R
Neurologische Klinik, Universität, Heidelberg.
Nervenarzt. 1996 Jun;67(6):502-5.
A case of acute microangiopathic hemolytic anemia in a 29-year-old primiparous patient is presented. Following an unsuspicuous pregnancy and uncomplicated delivery, the patient developed upper abdominal symptoms and transient psychopathologic abnormalities 2 days after delivery. Because of a series of generalized tonic-clonic seizures and respiratory distress she was admitted to the Neuro ICU. Focal neurological symptoms were absent, and CSF analysis was normal. Laboratory findings indicated hemolytic anemia with fragmented erythrocytes in smear specimens, thrombocytopenia, reticulocytosis, marked elevation of liver and pancreatic enzymes, and microhematuria. T2-weighted MR scans of the brain discluded multiple ischemic lesions of the cerebral white and gray matter, caudate nucleus and pons, displaying a hemodynamic pattern. Specific treatment included administration of corticosteroids, repeated plasmapheresis with substitution by fresh frozen plasma and antithrombin III. A control MR performed on the 3rd day of treatment demonstrated the absence of nearly all abnormal findings previously obtained. During a 3-week clinical course the patient showed full recovery, including normalization of hematologic and neuroradiologic findings. This case demonstrates ischemic cerebral lesions in acute microangiopathic hemolytic anemia to be potentially reversible if specific treatment is provided.
本文报告了一例29岁初产妇的急性微血管病性溶血性贫血病例。在经历了一次无异常的妊娠和顺产之后，患者在产后2天出现上腹部症状和短暂的精神病理异常。由于出现一系列全身性强直阵挛发作和呼吸窘迫，她被收入神经重症监护病房。未发现局灶性神经症状，脑脊液分析正常。实验室检查结果显示为溶血性贫血，涂片标本中有破碎红细胞、血小板减少、网织红细胞增多、肝酶和胰酶显著升高以及微量血尿。脑部T2加权磁共振成像排除了脑白质、灰质、尾状核和脑桥的多发缺血性病变，显示出一种血流动力学模式。具体治疗措施包括给予皮质类固醇、反复进行血浆置换并用新鲜冷冻血浆和抗凝血酶III替代。治疗第3天进行的对照磁共振成像显示几乎所有先前出现的异常表现均已消失。在为期3周的临床病程中，患者完全康复，血液学和神经放射学检查结果均恢复正常。该病例表明，如果给予特异性治疗，急性微血管病性溶血性贫血中的缺血性脑损伤可能是可逆的。
