成人斯蒂尔病伴皮病性淋巴结炎并发噬血细胞综合征1例报告及 文献复习

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[中图分类号] R593.2；R551.2 [文献标志码] B

ABSTRACTAdult-onset Stil's disease （AOSD） is a rare autoinflammatory disease characterized by fever，rash，arthritis，liver，spleen and lymph node enlargement，increased total number of peripheral white blood cells and neutrophil ratio.This paper reported a case of AOSD with dermal lymphadenitis （DL） complicated with hemophagocytic syndrome （HPS），in order to improve the clinicians’understanding for this complicated complication. The patient was a 48-year-old female who was admited to the hospital with the complant of“intermittent fever with rash for 15d′′ .After 10d of active anti-infection treatment，the symptoms were not improved，and there were new large congestive edematous erythema on the face and trunk，muscle pain in limbs and joints，and spleen enlargement. Laboratory tests showed increased white blood cell count，significantly decreased platelet count，hypofibrinogenemia，elevated serum ferrtin，and elevated soluble interleukin-2 receptor sCD25；DL was pathologically diagnosed by axillary lymph node biopsy.After excluding other diseases，the diagnosis was confirmed as AOSD with DL complicated with HPS.After diagnosis of HPS，the patient was treated with hemophagocytic lymphohistiocytosis （HLH）- 1994 regimen combined with rucotinib for 6 weeks，and the symptoms were improved；the patrent was discharged. The diagnosis of AOSD is particularly complex when complicated with the complications such asHPS，which requires carefully differential diagnosis，especially to exclude lymphoma.The cases of AOSD with DL are rare，and itsetiology and pathogenesis need further study；early diagnosis and multidisciplinary collaboration are essential to improve the patient’s prognosis.

KEYWORDsAdult-onset Stil's disease；Dermatopathic lymphadenitis；Hemophagocytic syndrome; Fever；Rash；Lymphadenectasis

成人斯蒂尔病（adult-onset Still's disease，AOSD）是一种以发热、皮疹、关节炎或关节痛、咽痛、肝脾和淋巴结肿大、外周血白细胞总数及中性粒细胞比例增高等为主要表现的，少见且病因不明的全身性自身炎症性疾病[1]。（剩余10695字）