成人原发性皮肤T淋巴母细胞性淋巴瘤1例报告并文献复习

Adult Primary Cutaneous T-Lymphoblastic Lymphoma:A Case Report and Review of Literature

目的了解成人原发性皮肤T淋巴母细胞性淋巴瘤(T-LBL)的临床、诊断、治疗及预后,提高对本病的认识。方法对南昌大学第一附属医院2014年5月收治的1例成人皮肤T淋巴母细胞性淋巴瘤患者的临床资料结合相关文献进行回顾性分析。结果该例患者以皮肤结节为首发症状,病理及免疫组化确诊为T淋巴母细胞性淋巴瘤。按CHOP样化疗方案间断治疗14次,病情反复,最终浸润骨髓,乳酸脱氢酶(LDH)逐渐升高,于2016年5月因呼吸循环衰竭死亡(考虑肺栓塞)。自发病至死亡时间约2年。结论 T淋巴母细胞性淋巴瘤的皮肤损害极少见,属于高度侵袭性淋巴瘤,生存期短。LDH水平升高可能预示预后不良。

Objective To investigate the clinical features,diagnosis,treatment and prognosis of primary cutaneous T-lymphoblastic lymphoma(T-LBL)in adults,and to raise awareness of this disease.Methods Clinical data of an adult cutaneous T-LBL patient treated in the First Affiliated Hospital of Nanchang University in May 2014 was analyzed retrospectively,and the related literature was reviewed.Results Cutaneous nodule was the first symptom of T-LBL in this patient,which was confirmed by pathology and immunohistochemistry.The patient was intermittently treated 14 times with CHOP-like chemotherapy.However,relapse repeatedly occurred and finally resulted in bone marrow infiltration and gradual increase in lactic dehydrogenase(LDH)levels.The patient died of respiratory failure in May 2016(pulmonary embolism was considered).The duration between disease onset and death was about 2 years.Conclusion T-LBL rarely involves the skin.It belongs to the highly aggressive lymphoma and is associated with short overall survival.The increase in LDH levels may indicate poor prognosis.