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 recogni-tion 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 Lympho-proliferative 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.