| 张亚华 张璇 唐海龙 曹春 陈协群 高广勋.系统性种痘水疱病样皮肤T 细胞淋巴瘤1 例及文献复习[J].,2015,15(25):4892-4895 |
| 系统性种痘水疱病样皮肤T 细胞淋巴瘤1 例及文献复习 |
| Hydroa Vacciniforme-like Cutaneous T Cell Lymphoma: a Case Report andLiterature Review |
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| DOI： |
| 中文关键词: 种痘水疱病 淋巴瘤 T细胞淋巴瘤 EBV 阳性 |
| 英文关键词: Hydroa vacciniforme Lymphoma Epstein-Barr virus positive |
| 基金项目:家自然科学基金项目（30900639）；陕西省自然科学基金项目（2014JM2-8161） |
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| 中文摘要: |
| 目的：通过分析系统性种痘水疱病样淋巴瘤病例1 例，结合文献回顾，分析其临床特点、诊断治疗进展及预后，以提高临床
医生对该病的认识。方法：报道1 例有7 年种痘水疱病史并转化为系统性T 细胞淋巴瘤病例，通过皮肤活检、病理及免疫组化、
TCR基因重排、实验室、MRI及影像学检查，确定诊断及治疗方案，并观察预后。后进行文献回顾。结果：本例患者的主要临床表现
为多年的面部红斑、丘疱疹及水疱改变，最初诊断为" 种痘水疱病"，初期治疗有效，病情反复并进展，皮肤改变加重，病变逐渐累
及鼻中隔及下鼻甲，出现坏死及缺损，同时伴有发热及淋巴结肿大等系统症状。皮肤病理及免疫组化提示真皮弥漫性淋巴细胞、
浆细胞浸润，CD3+，CD4+，CD8 散在细胞+，CD30 局部细胞+，CD56 局部细胞+，EBER 杂交（+），Ki-67增殖指数为60 %。TCR基
因克隆性重排。经干扰素及激素治疗初期病情控制尚可，持续约7 年时间，后病情进行性加重，表现系统性种痘水疱病样皮肤T
细胞淋巴瘤症状，给予MESA 方案化疗1 次。化疗后病情稳定，鼻部症状明显改善。后病情进展，患者出现神经系统症状并死亡。
结论：种痘水疱病样淋巴瘤早期临床表现易与种痘样水疱病相混淆，但根据其病变部位病理活检及免疫组化分析可得到确诊，早
期单纯皮肤病变期可持续数年，部分对激素及干扰素治疗有效。当发展为系统性种痘水疱病样淋巴瘤期时，病情进展迅速，常可
累及中枢神经系统，对于化疗反应差，预后不佳，死亡率极高。化疗对于该病的有效性有待进一步观察。 |
| 英文摘要: |
| Objective:To report and analyze a case of hydroa vacciniforme-like lymphoma to review the pathology, clinical
features, diagnosis, treatment and prognosis of this kind of lymphoma, in order to improve its understanding for clinic.Methods:Reported
a case with 7 years history of Hydroa Vacciniforme and translated into systemic T-cell lymphoma cases finally, through the skin biopsy,
pathologic and immunohistochemical, TCR gene rearrangement, laboratory test, MRI and imaging examination, we made a clinical
diagnosis and treatment plan, then to find outcome. The final is the literature review.Results:The clinical features of this case are facial
edema, papulovesicles and blister, nasal septum and inferior nasal concha were involved. At the same time, the patient had fever,
lymphadenopathy. Monoclonal T cell receptor (TCR)-gamma gene rearrangement in the skin biopsy, lymphocytes and plasma cells
infiltrated in the dermis, CD3+, CD4+, CD8+, CD30+, CD56 +, EBER (+）, MIB-1 60 %. With treatment of interferon in the early stage, it
could be controlled well for 7 years. After that disease progressed, the patient was diagnosed Hydroa vacciniforme-like cutaneous T cell
lymphoma and accepted MESA chemotherapy. Finally, she presented the symptoms of central nervous system and died.Conclusion:In
the early stage, hydroa vacciniforme-like lymphoma is resembled to hydroa vacciniforme very much. It can be confirmed by pathological
biopsy and immunohistochemical analysis. This disease progresses fast and has poor prognosis, parts of the patients are effective to
interferon and steroid, but the effect of chemotherapy is still unclear. |
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