Hemophagocytic lymphohistiocytosis triggered by relapsing polychondritis:A case report  

作　　者：Mi-Ran Han Jeong-Hwan Hwang Seungah Cha So-Yeon Jeon Kyu Yun Jang Namsu Kim Chang-Hoon Lee 

机构地区：[1]Department of Internal Medicine,Jeonbuk National University Medical School,Jeonju 54907,South Korea [2]Department of Pathology,Jeonbuk National University Medical School,Jeonju 54907,South Korea [3]Department of Laboratory Medicine,Jeonbuk National University Medical School,Jeonju 54907,South Korea

出　　处：《World Journal of Orthopedics》2024年第8期813-819,共7页世界骨科杂志（英文版）

摘　　要：BACKGROUND Hemophagocytic lymphohistiocytosis(HLH)is a rare,life-threatening disorder caused by abnormal histiocytes and T cell activation.In adults,it is predominantly associated with infections,cancers,and autoimmune diseases.Relapsing polychondritis(RP),another rare disease,is diagnosed based on symptoms without specific tests,featuring cartilage inflammation characterized by swelling,redness,and pain,rarely inducing HLH.CASE SUMMARY A 74-year-old woman visited the emergency room with a fever of 38.6℃.Blood tests,cultures,and imaging were performed to evaluate fever.Results showed increased fluorescent antinuclear antibody levels and mild cytopenia,with no other specific findings.Imaging revealed lymph node enlargement was observed;however,biopsy results were inconclusive.Upon re-evaluation of the physical exam,inflammatory signs suggestive of RP were observed in the ears and nose,prompting a tissue biopsy for confirmation.Simultaneously,persistent fever accompanied by cytopenia prompted a bone marrow examination,revealing hemophagocytic cells.After finding no significant results in blood culture,viral markers,and tissue examination of enlarged lymph nodes,HLH was diagnosed by RP.Treatment involved methylprednisolone followed by azathioprine.After two months,bone marrow examination confirmed resolution of hemophagocytosis,with normalization of hyperferritinemia and pancytopenia.CONCLUSION Thorough physical examination enabled diagnosis and treatment of HLH trig gered by RP in patients presenting with fever of unknown origin.

关 键 词：Hemophagocytic lymphohistiocytosis Relapsing polychondritis Autoimmune disease Fever of unknown origin STEROID Case report 

分 类 号：R681.3[医药卫生—骨科学] R73[医药卫生—外科学]