Pathological type composition of lymphoid tissue diseases with hemophagocytic lymphohistiocytosis manifestations:analyses of 327 cases
ZHENG Xiao-dan
XIE Jian-lan
ZHENG Yuan-yuan
TENG Xiao-jing
ZHANG Yan-lin
Abstract：Objective To analyze the types of lymphoid tissue diseases with hemophagocytic lymphohistiocytosis(HLH)manifestations and the percentage of their composition in order to improve the understanding of this group of diseases.Methods A total of 327 patients with secondary HLH who underwent external examination and consultation at Beijing Friendship Hospital of Capital Medical University from 2017 to 2024 were collected,and their gender,age,and primary disease type were analyzed.Results Among the 327 patients,there were 196 males and 131 females,with a male to female ratio of 1.5∶1.The age ranged from 6 months to 75 years,with a median age of 14 years and an average age of 24.5 years.219 cases showed hematoophagy,including 133 cases in the bone marrow,49 cases in the lymph nodes,32 cases in the spleen,and 5 cases in the liver As for the primary diseases,there were 185 cases(56.6%)of EBV+T/NK cell lymphoid tissue proliferation and lymphoma,27 cases(8.3%)of other EBV-associated lymphomas,49 cases of non-EBV infection-associated lymphomas and 66 other non-neoplastic diseases among the 327 patients.EBV test showed that a total of 251 cases(76.8%)were associated with EBV infection,with the highest number of cases being systemic chronic active EBV infection(133 cases,53.0%),followed by infectious mononucleosis(39 cases,15.5%).Conclusion EB virus-positive lymphoid tissue diseases,especially those related to T/NK cell infection,are important causes of HLH.Early and accurate identification of the specific types of EB virus-positive lymphoid tissue diseases may help to further precise clinical management.
Keywords：Hemophagocytic lymphohistiocytosisLymphomaEpstein-Barr virusHematophagyPathology
Publication Date：2025-06-28
Online Publishing Date：2025-08-15(First online date of this platform, not the publication date of the document)
Pages：5( 643-647 )