自体外周血造血干细胞移植治疗原发皮肤γδT细胞淋巴瘤

Autologous Peripheral Blood Hematopoietic Stem Cell Transplantation for Treating Patient with Primary Cutaneous γδT Cell Lymphoma

目的:探讨原发皮肤γδT细胞淋巴瘤的临床特点和治疗方法。方法:对在2013年7月我科确诊的1例原发皮肤γδT细胞淋巴瘤患者的临床资料和治疗经过进行分析。患者系39岁女性,以多发皮下结节为主要临床特点,皮肤活检病理检查免疫表型为CD20^-、CD3^+、CD4^-、CD8^-、CD56^+、TIA^-1^+、Ki^-67^+(约60%);浆细胞κ^+(部分)/λ^+(部分);组织细胞CD4^+、CD68/PGM1^+;βF1^-、EB病毒EBER原位杂交检查阴性。结果:给予包含左旋门冬酰胺酶的化疗方案获得疾病缓解,于2014年2月行自体造血干细胞移植术,术后14 d粒细胞稳定植入,12 d血小板稳定植入。术后随访,缓解至今。结论:原发皮肤γδT细胞淋巴瘤临床罕见,容易误诊,疾病呈侵袭性,临床进展快,应用含左旋门冬酰胺酶的化疗方案治疗有一定疗效,自体或异基因造血干细胞移植术有助于延长患者生存。

Objective: To investigate the clinical characteristics of primary cutaneous γδ T cell lymphoma and its treatment methods. Methods: The clinical data and treatment process of one w oman case of primary cutaneous γ δ T cell lymphoma diagnosed in our department w ere analysed. The multiple subcutaneous nodules w ere the main clinical features,the diagnosis of primary cutaneous γδ T cell lymphoma w as comfired by skin biopsy pathology. The immunophenotypes of lymphocytes show ed CD20^-,CD3^+,CD4^-,CD8^-,CD56^+,TIA^-1^+,Ki^-67^+( about 60%); plasma cells kappa ^+( part) /lambda predominate ^+( part); histocytes CD4^+,CD68 /PGM1^+; βF1^-,epstein^-barr( EB) virus showed negative EBER in situ hybridization. Results: By means of the chemotherapy regimens containing L^-Asparaginase,the complete remission( CR) w as achieved. Then,the patients w ere given autologous hematopoietic stem cell transplantation. Neutrophils w ere implanted after 16 days,and platelet w as implanted after 18 days. Now,the patient is still in remission. Conclusion:primary cutaneous γδ T cell lymphoma is rare and easy to be misdiagnosed. This disease is aggressive and its prognosis is poor. The large dose chemotherapy w ith L^-asparaginase show s a certain curative efficacy,the autologous hematopoietic stem cells can prolong survival time of the patient.