慢性移植物抗宿主病并发硬皮病样综合征1例

Q3 Medicine
I. Parvova, V. Taneva, E. Ivanova-Todorova, D. Kyurkchiev, Z. Kolarov, E. Hristov
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引用次数: 1

摘要

异基因造血干细胞移植后的前100天内观察到慢性移植物抗宿主病,可影响所有组织和器官(80%的病例影响皮肤)。慢性移植物抗宿主病与某些自身免疫性疾病之间存在一些临床对应关系,如系统性硬皮病、干燥综合征、自身免疫性肝炎。我们报告了一例54岁的男性,其表现为弥漫性硬皮病样皮肤变化,该变化发生在无关供体的同种异体骨髓移植后约一年半,原因是2型慢性粒单核细胞性白细胞病的爆炸性转化。患者在血液学诊所接受了皮质类固醇治疗,10次光泳疗程,环孢素、他克莫司、霉酚酸酯、伊马替尼。据推测,这是一种对皮质类固醇具有耐药性的慢性移植物抗宿主疾病,每隔一个月开一次甲氨蝶呤25mg/周,但没有显著的临床改善。就鉴别诊断而言,问题仍然是这是否是副肿瘤性系统性硬皮病——伴随恶性肿瘤的自身免疫现象,通常会持续数月。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
A clinical case of a patient with scleroderma-like syndrome in chronic graft-versus host disease
Chronic graft versus host disease is observed within the fi rst 100 days following allogeneic haematopoietic stem cell transplantation and can affect all tissues and organs (in 80% of the cases, it affects the skin). There are some clinical correspondences between chronic graft versus host disease and certain autoimmune diseases, such as systemic scleroderma, Sjogren‘s syndrome, autoimmune hepatitis. We present a case of a 54-year-old man with manifested diffuse, scleroderma-like skin changes, which occurred about a year and a half after allogenic bone marrow transplantation from an unrelated donor due to a blast transformation of chronic myelomonocytic leucosis type 2. The patient was treated in a haematology clinic with corticosteroids, 10 photophoresis sessions, Ciclosporin, Tacrolimus, Mycophenolate mofetil, Imatinib. It has been assumed that this is a case of chronic graft versus host disease resistant to corticosteroids, and Methotrexate 25 mg/weekly every other month was prescribed with no significant clinical improvement. In terms of the differential diagnosis, the question remains whether or not this is a case of paraneoplastic systemic scleroderma – autoimmune phenomena accompanying malignancy and often preceding it for months.
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来源期刊
Revmatologiia (Bulgaria)
Revmatologiia (Bulgaria) Medicine-Rheumatology
CiteScore
0.30
自引率
0.00%
发文量
21
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