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[特发性血小板减少性紫癜治疗期间的急性播散性脑脊髓炎]

[Acute disseminated encephalomyelitis during treatment for idiopathic thrombocytopenic purpura].

作者信息

Hoshino Takumi, Hatsumi Nahoko, Takada Satoru, Sakura Tohru, Sakurai Atsushi, Miyawaki Shuichi

机构信息

Leukemia Research Center, Saiseikai Maebashi Hospital.

出版信息

Rinsho Ketsueki. 2008 Jul;49(7):505-9.

Abstract

A 54-year-old woman had an episode of sudden oral bleeding and generalized petechiae 1 week after a sore throat and diarrhea. On admission, the platelet count was 0.1 x 10(4)/microl, and the platelet-associated IgG level was elevated. Hyperplasia of megakaryocytes in a bone marrow specimen and aberrant Epstein-Barr virus (EBV) antibody patterns led to a diagnosis of EBV-associated idiopathic thrombocytopenic purpura (ITP). Prednisolone (PSL) promptly restored her platelet count; however, she developed disorientation and affective lability soon after PSL was tapered. Subsequently, she ran a high fever and developed convulsive seizures. T2-weighted MRI demonstrated a high signal area in the subcortical white matter, and no abnormal findings were found on examination of the cerebrospinal fluid. The diagnosis of acute disseminated encephalomyelitis (ADEM) was made and steroid pulse therapy was started, which resulted in remission of the symptoms without recurrence in the following months. This is the first reported case of ADEM following EBV infection during treatment for ITP. Administration of PSL for ITP might mask the presenting clinical picture of ADEM. The possibility of ADEM should be investigated in patients of ITP following viral infection who develop acute encephalopathy.

摘要

一名54岁女性在出现咽痛和腹泻1周后,突发口腔出血和全身瘀点。入院时,血小板计数为0.1×10⁴/微升,血小板相关IgG水平升高。骨髓标本中巨核细胞增生以及异常的EB病毒(EBV)抗体模式导致诊断为EBV相关的特发性血小板减少性紫癜(ITP)。泼尼松龙(PSL)迅速恢复了她的血小板计数;然而,在PSL减量后不久,她出现了定向障碍和情感不稳。随后,她高热并出现惊厥发作。T2加权磁共振成像(MRI)显示皮质下白质有高信号区,脑脊液检查未发现异常。诊断为急性播散性脑脊髓炎(ADEM)并开始进行类固醇冲击治疗,症状缓解,在接下来的几个月中未复发。这是首例关于ITP治疗期间EBV感染后发生ADEM的报道病例。ITP使用PSL治疗可能掩盖ADEM的临床表现。对于病毒感染后发生急性脑病的ITP患者,应调查其发生ADEM的可能性。

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