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[脐带血移植后发生环孢素A诱导的脑病的骨髓增生异常综合征,表现为对右侧基底节区的占位效应]

[Myelodysplastic syndrome with cyclosporine A-induced encephalopathy presenting with a mass effect on the right basal ganglia after cord blood transplantation].

作者信息

Koh Hideo, Yamane Takahisa, Nakane Takahiko, Takeoka Yasunobu, Sakamoto Erina, Kanashima Hiroshi, Nakamae Mika, Nakamae Hirohisa, Koh Ki-Ryang, Mochizuki Kunizo, Hayashi Noriko, Hino Masayuki

机构信息

Clinical Hematology and Clinical Diagnostics, Graduate School of Medicine, Osaka City University.

出版信息

Rinsho Ketsueki. 2006 Dec;47(12):1539-43.

Abstract

An 18-year-old female with myelodysplastic syndrome underwent an allogeneic cord blood transplantation in May 2005. The conditioning regimen consisted of total body irradiation, cytarabine and cyclophosphamide. The day of the cord stem cell transfusion was counted as Day 0. For acute GVHD prophylaxis, cyclosporine A (CsA) and methotrexate were used. Engraftment was achieved on Day 30, acute GVHD grade II developed on Day 45 and treatment with methylprednisolone for acute GVHD was started. On Day 68 the patient had generalized seizures accompanied by loss of consciousness, hypertension and left hemiparesis, and was intubated. A cranial CT scan showed a mass effect on the right basal ganglia, and high signal intensities on the T2-weighted and FLAIR images of a MR examination were detected in the bilateral basal ganglia and posterior lobes, the findings of which were compatible with a brain tumor or infectious disease. Since an increased level of apparent diffusion coefficient (ADC) values on the bilateral basal ganglia was noted, we suspected that vasogenic edema had caused the mass effect. She went into remission immediately after CsA treatment was discontinued. Therefore, this case was diagnosed as atypical reversible posterior leukoencephalopathy syndrome (RPLS) associated with CsA. CsA-induced encephalopathy presenting a mass effect in clinical imaging findings is very rare, and an ADC map may be useful for the diagnosis of RPLS.

摘要

一名18岁的骨髓增生异常综合征女性患者于2005年5月接受了异基因脐血移植。预处理方案包括全身照射、阿糖胞苷和环磷酰胺。脐血细胞输注日计为第0天。为预防急性移植物抗宿主病(GVHD),使用了环孢素A(CsA)和甲氨蝶呤。在第30天实现造血重建,在第45天出现II级急性GVHD,并开始用甲泼尼龙治疗急性GVHD。在第68天,患者出现全身性癫痫发作,伴有意识丧失、高血压和左侧偏瘫,并进行了气管插管。头颅CT扫描显示右侧基底节有占位效应,磁共振检查的T2加权像和液体衰减反转恢复序列(FLAIR)图像上双侧基底节和后叶检测到高信号强度,这些表现与脑肿瘤或感染性疾病相符。由于双侧基底节表观扩散系数(ADC)值升高,我们怀疑血管源性水肿导致了占位效应。在停用CsA治疗后,她立即病情缓解。因此,该病例被诊断为与CsA相关的非典型可逆性后部白质脑病综合征(RPLS)。CsA诱导的脑病在临床影像学表现中呈现占位效应非常罕见,ADC图可能有助于RPLS的诊断。

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