免疫缺陷相关淋巴组织增殖性疾病临床病理特征分析  

Clinicopathological features of lymphoproliferative diseases associated with immune deficiency

作　　者：王小树 李君娜 于靖雯 马文梅 崔文丽[1] WANG Xiao-shu;LI Jun-na;YU Jing-wen;MA Wen-mei;CUI Wen-li(Department of Pathology,the First Affiliated Hospital of Xinjiang Medical University,Urumqi 830000,China)

机构地区：[1]新疆医科大学第一附属医院病理科,乌鲁木齐830000

出　　处：《诊断病理学杂志》2024年第9期830-833,共4页Chinese Journal of Diagnostic Pathology

基　　金：国家自然科学基金地区科学基金项目(82060038,82360046);CSCO恒瑞肿瘤研究基金项目面上课题(YHR2020QN-0153);天山英才医药卫生高层次人才培养计划(TSYC202301A009)。

摘　　要：目的 分析免疫缺陷相关淋巴组织增殖性疾病(LPD)的临床和病理特点,复习国内外相关文献,加深对其理解和认识。方法 回顾性分析淋巴结及相关受累组织活检明确诊断的免疫缺陷相关淋巴组织增殖性疾病的形态学、病理诊断、间隔时间、主要治疗方法及预后。结果 纳入病例10例,移植后淋巴组织增殖性疾病(PTLD)8例,多形性免疫失调相关淋巴组织增殖性疾病1例、HIV感染相关淋巴组织增殖性疾病1例。PTLD有非破坏型、多形性、单形性和经典霍奇金型。免疫缺乏特异性,EBER阳性。结论 免疫缺陷相关淋巴组织增殖性疾病可累及淋巴结及结外器官,形态学是其诊断的重要方法,治疗主要在于提高免疫力、合理使用化疗药物、手术及抗病毒。Objective To analyze the clinical and pathological characteristics of lymphoproliferative diseases associated with immune deficiency with review of the relevant literatures at home and abroad to deepen the understanding and recognition of lymphoproliferative diseases.Methods The morphology,pathological diagnosis,interval,main treatment methods and prognosis of patients with lymphoproliferative diseases associated with immune deficiency confirmed by biopsy of lymph nodes and related involved tissues were retrospectively analyzed.Results In 10 cases,transplantation lymphoproliferative hyperplastic diseases(PTLD)were 8 cases,pleomorphic immune dysregulation related lymphoproliferative diseases was 1 case,and HIV infection-related lymphoproliferative diseases 1 case.PTLD included non-destructive,pleomorphic,monomorphic and classical Hodgkin types.Immunodeficiency lacked specificity,EBER was positive.Conclusion Lymphoproliferative diseases associated with immune deficiency may involve lymph nodes and extranodal organs.Morphology is an important method of diagnosis,and treatment is mainly to improve immunity,rational use of chemotherapy drugs,surgery and antiviral therapy.

关 键 词：免疫缺陷相关淋巴组织增殖性疾病 临床病理特征 移植后 EB病毒治疗 

分 类 号：R733.41[医药卫生—肿瘤]