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自体骨髓移植后播散性浅表性汗孔角化症

Disseminated superficial porokeratosis after autologous bone marrow transplantation.

作者信息

Rio B, Magana C, Le Tourneau A, Bachmeyer C, Lévy V, Hamont N, Diebold J, Zittoun R

机构信息

Service d'Hématologie, Hôtel-Dieu de Paris, France.

出版信息

Bone Marrow Transplant. 1997 Jan;19(1):77-9. doi: 10.1038/sj.bmt.1700546.

Abstract

A case of disseminated superficial porokeratosis (DSP) is reported in a black man 5 years after autologous bone marrow transplantation (BMT) for acute promyelocytic leukemia. Porokeratosis is a rare hyperkeratotic disorder arising from clonal keratinocytes with a high potential to develop squamous cell carcinoma. Inherited forms are classical but recent observations of acquired porokeratosis have been reported in immunocompromized patients (AIDS, immune disorders, immune suppressive drugs or organ transplantation). Two cases of DSP have been reported after allogeneic BMT in patients treated for chronic GVHD. Our case is the first one after autologous BMT, in a black man, on no immunosuppressive drug at the time of diagnosis of DSP. Hematopoietic and immune reconstitution was apparently complete. The cancer-prone character of porokeratosis could be favored by total body irradiation used in conditioning regimen. Thus, porokeratosis has to be associated with other late effects after BMT such as HCV seropositivity, cataract and infertility that were observed in this patient.

摘要

一名黑人男性在接受自体骨髓移植(BMT)治疗急性早幼粒细胞白血病5年后,出现了播散性浅表性汗孔角化症(DSP)病例。汗孔角化症是一种罕见的角化过度性疾病,由具有高发展为鳞状细胞癌潜能的克隆性角质形成细胞引起。遗传性形式较为典型,但最近在免疫功能低下的患者(艾滋病、免疫紊乱、免疫抑制药物或器官移植)中报告了获得性汗孔角化症的观察结果。在接受慢性移植物抗宿主病(GVHD)治疗的患者中,异基因BMT后已报告了2例DSP病例。我们的病例是自体BMT后首例,患者为黑人男性,诊断DSP时未使用免疫抑制药物。造血和免疫重建显然已完成。预处理方案中使用的全身照射可能会促进汗孔角化症的致癌特性。因此,汗孔角化症必须与该患者中观察到的BMT后的其他晚期效应相关联,如丙型肝炎病毒血清阳性、白内障和不育症。

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