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目的:探讨蕈样霉菌病(MF)的临床病理特点、鉴别诊断、治疗及预后。方法:分析24例MF(包括6例斑片期、11例斑块期和7例肿瘤期)患者的临床资料、病理形态、免疫组化染色、治疗方案及预后。结果:24例MF患者中,男女比为1.7∶1,平均年龄为52岁。临床表现多数符合经典的斑片、斑块至肿瘤期的演变过程。病理表现为真皮内具有脑回状细胞核的异型T淋巴细胞呈灶性、片状或弥漫性浸润,可累及皮下脂肪组织,可见亲表皮性及Pautrier微脓肿。免疫组化肿瘤细胞表达CD3、CD45RO、CD4。早期患者经皮肤靶向治疗及生物学治疗,病情可部分缓解,2例晚期患者给予联合化疗。随访患者中仅有2例在7个月内死亡(平均随访时间1.9年)。结论:MF是皮肤T细胞淋巴瘤中最常见的类型,患者临床表现多样,诊断主要依赖临床特点、病理组织学和免疫组化检查结果。
Objective: To investigate the clinicopathological features, differential diagnosis, treatment and prognosis of mycosis fungoides (MF). Methods: The clinical data, pathomorphology, immunohistochemical staining, treatment and prognosis of 24 patients with MF (including 6 cases of plaque, 11 cases of plaque and 7 cases of tumor) were analyzed. Results: Among 24 MF patients, the ratio of men to women was 1.7: 1, with an average age of 52 years. Most of the clinical manifestations of the classic patch, plaque to the evolution of the tumor stage. Pathological manifestations of the dermis with retromorphic brain nuclei of T-lymphocytes were focal, flaky or diffuse infiltration, can affect subcutaneous adipose tissue, showing pro-epidermal and Pautrier micro abscess. Immunohistochemical tumor cells expressed CD3, CD45RO, CD4. Early skin-targeted therapy and biological treatment of patients, the disease can be partially relieved, two cases of advanced patients given combined chemotherapy. Only 2 of the patients who were followed died within 7 months (mean follow-up, 1.9 years). Conclusion: MF is the most common type of cutaneous T-cell lymphoma. The clinical manifestations of the patients are diverse. The diagnosis depends mainly on the clinical features, histopathology and immunohistochemistry findings.