以皮肤损害为首发症状的鼻型结外NK/T细胞淋巴瘤患者的临床分析  

Clinical Analysis of Extranodal NK/T-Cell Lymphoma,Nasal Type with Skin Lesions as Initial Symptom

作　　者：程平[1] 李义[2] 毛霞[2] 王秋香 王兰兰 关军[1] 周英[1] 程辉[1] CHENG Ping;LI Yi;MAO Xia;WANG Qiu-Xiang;WANG Lan-Lan;GUAN Jun;ZHOU Ying;CHENG Hui(Department of Hematology,Wuhan First Hospital;Department of Hematology,Tongji Hospital,Tongji Medical College,Huazhong University of Science and Technology,Wuhan 430022,Hubei Province,China)

机构地区：[1]武汉市第一医院血液内科 [2]华中科技大学同济医学院附属同济医院血液科,湖北武汉430022

出　　处：《中国实验血液学杂志》2025年第2期416-422,共7页Journal of Experimental Hematology

摘　　要：目的:探讨以皮肤损害为首发症状的鼻型结外NK/T细胞淋巴瘤(ENKTL)患者的临床特征、治疗方案及预后。方法:回顾性分析2016年8月至2023年1月在武汉市第一医院和华中科技大学同济医学院附属同济医院诊治的11例以皮肤损害为首发症状的ENKTL患者的临床资料。结果:11例患者中,男性6例,女性5例,中位年龄50(32-80)岁。起病时均以不同形式的皮肤损害为首发临床症状,表现为皮疹、溃疡性肿块、皮肤痛性结节、浸润性斑疹等,皮损累及部位多为四肢及躯干,也可单独出现于下肢。5例初诊时合并噬血细胞综合征(HLH),8例存在B症状。所有患者诊断时临床分期晚(Lugano分期Ⅳ期),危险分层为高危(PINK-E评分≥3分)。病损皮肤组织免疫组织化学检查CD56、EBER阳性率均为100%,中位Ki-67指数75%(50%-80%)。血浆EBV-DNA检测均为阳性(≥5×10^(2) copies/ml)。诱导化疗方案多为含培门冬酶或左旋门冬酰胺酶的联合化疗(MESA、p-Gemox、SMILE)或联合PD-1单抗免疫治疗,或选择HLH的方案(HLH-04方案、L-DEP)治疗。中位随访时间及总生存时间均为4.5(0.5-27)个月,随访期内未接受自体造血干细胞移植(ASCT)治疗的8例患者全部死亡,死因多为疾病快速进展。3例患者接受了ASCT治疗,1例移植后中枢神经系统复发死亡,2例存活。接受ASCT的3例患者OS分别为21、27和19个月,PFS分别为11、20和13个月。移植后不定期监测血浆EBV-DNA拷贝数,EBV的载量与病情的变化呈现一致性。结论:以皮肤损害为首发症状的ENKTL患者早期临床症状更不典型,早期诊断尤为困难,疾病进展迅速,预后很差,最佳治疗策略仍无统一标准。大剂量诱导化疗获得完全缓解后尽快行ASCT可使患者生存期明显延长。Objective:To investigate the clinical features,treatment and prognosis of extranodal NK/T-cell lymphoma,nasal type(ENKTL)with skin lesions as initial symptom.Methods:The clinical data of 11 ENKTL patients with skin lesions as initial symptom were retrospectively analyzed from August 2016 to January 2023 in Wuhan First Hospital and Tongji Hospital,Tongji Medical College,Huazhong University of Science and Technology.Results:Among the 11 patients,there were 6 males and 5 females,with a median age of 50(32-80)years.All patients had different forms of skin lesions as initial clinical symptom,including rash,ulcerative mass,painful skin nodules,infiltrating macula,etc.Most of the skin lesions were involved in the limbs and trunk but also appeared in the lower limbs alone.Five patients had hemophagocytic lymphohistiocytosis(HLH)at initial diagnosis,and 8 patients had B symptoms.All patients were diagnosed with advanced clinical staging(Lugano staging IV),and classified as high risk(PINK-E score≥3).Immunohistochemical examination revealed that the positive rates of CD56 and EBER were both 100%,and the median Ki-67 index was 75%(50%-80%).Plasma EBV-DNA tests were all positive(≥5×10^(2) copies/ml).Most of the induction chemotherapy regimens were combination chemotherapy(MESA,p-Gemox,SMILE)containing pegaspargase or L-asparaginase,or combined with PD-1 monoclonal immunotherapy,or HLH regimens(HLH-04 regimen,L-DEP).The median follow-up time and overall survival(OS)time were both 4.5(0.5-27)months.During the follow-up period,all 8 patients who did not receive autologous hematopoietic stem cell transplantation(ASCT)died,most of whom died of rapid disease progression.Three patients received ASCT,one died of central nervous system recurrence after transplantation,and two survived.The OS of three patients who underwent ASCT was 21,27,and 19 months,and PFS was 11,20,and 13 months,respectively.The plasma EBV-DNA copy number was monitored irregularly after transplantation,and the load of EBV was consistent with the changes of th

关 键 词：鼻型结外NK/T细胞淋巴瘤 皮肤损害 化疗 造血干细胞移植 

分 类 号：R733.1[医药卫生—肿瘤]