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目的 探讨组织细胞吞噬性脂膜炎(CHP)的临床特点,以提高对CHP的认识。方法分析我院1994年以来确诊的6例CHP的临床资料,并进行相关文献复习。结果 (1)6例患者中5例为女性,年龄8～47岁,病程1-24个月;(2)临床表现主要为发热(6/6)、皮下结节(6/6)、脾大(5/6)和肝大(4/6),实验室检查以肝酶升高(4/6)、凝血功能异常(2/4)、血细胞减少(3/6)多见;(3)病理学检查在皮下脂肪组织(6/6)及骨髓组织(3/3)可见形态正常的组织细胞浸润,并见吞噬血细胞现象;(4)仅1例患者采用联合化疗方案。结论 CHP具有独特的临床特点,诊断依赖于病理学,而积极联合化疗对改善预后有利。
Objective To investigate the clinical features of tissue phagocytic panniculitis (CHP) in order to improve the understanding of CHP. Methods The clinical data of 6 cases of CHP diagnosed in our hospital since 1994 were analyzed and the related literatures were reviewed. Results (1) Five of the 6 patients were female, aged from 8 to 47 years and had a duration of 1-24 months. (2) The clinical manifestations were mainly fever (6/6), subcutaneous nodules (6/6), spleen (5/6) and liver enlargement (4/6). Laboratory tests showed elevated liver enzymes (4/6), abnormal coagulation (2/4) and cytopenias (3/6) ) Pathological examination of subcutaneous adipose tissue (6/6) and bone marrow (3/3) showed normal morphological cell infiltration, and see the phenomenon of phagocytosis; (4) only 1 patient with combination chemotherapy. Conclusion CHP has a unique clinical features, the diagnosis depends on the pathology, and active combination chemotherapy is beneficial to improve the prognosis.