Ichikawa Satoshi, Fukuhara Noriko, Hatta Shunsuke, Himuro Masahito, Katsushima Hiroki, Nasu Kentaro, Ono Koya, Inokura Kyoko, Kobayashi Masahiro, Onishi Yasushi, Fujii Hiroshi, Ishizawa Kenichi, Ichinohasama Ryo, Harigae Hideo
Department of Hematology and Rheumatology, Tohoku University Hospital, Japan.
Department of Hematopathology, Tohoku University Hospital, Japan.
Intern Med. 2018 Jul 15;57(14):2051-2055. doi: 10.2169/internalmedicine.0568-17. Epub 2018 Mar 9.
A 16-year-old boy, who had been initially examined for bilateral blepharedema and slight eruption, presented with rapidly deteriorating symptoms in associating with headache and consciousness disturbance. He was diagnosed to have primary cutaneous CD8-positive aggressive epidermotropic cytotoxic T-cell lymphoma (PCAE-CTL) by a biopsy of the skin and brain. After whole-brain radiation and some courses of chemotherapy, cord blood transplantation was performed with myeloablative conditioning. After transplantation, the cerebral dysfunction gradually improved. Disease remission was confirmed by the disappearance of any abnormal findings on electroencephalogram and magnetic resonance imaging. PCAE-CTL is reported to be an extremely aggressive disease with a poor prognosis, but the timely performance of cord blood transplantation is considered to be a promising treatment strategy.
一名16岁男孩,最初因双侧眼睑水肿和轻微皮疹接受检查,随后出现与头痛和意识障碍相关的症状迅速恶化。通过皮肤和脑部活检,他被诊断为原发性皮肤CD8阳性侵袭性亲表皮细胞毒性T细胞淋巴瘤(PCAE-CTL)。在进行全脑放疗和几个疗程的化疗后,采用清髓性预处理进行了脐血移植。移植后,脑功能障碍逐渐改善。脑电图和磁共振成像上任何异常表现的消失证实了疾病缓解。据报道,PCAE-CTL是一种极具侵袭性、预后不良的疾病,但及时进行脐血移植被认为是一种有前景的治疗策略。