组织细胞性吞噬性脂膜炎

Hilton D A, O'Malley B P, Durrant S T

Department of Histopathology, Leicester Royal Infirmary, Infirmary Square, UK.

Postgrad Med J. 1990 Nov;66(781):958-61. doi: 10.1136/pgmj.66.781.958.

A 33 year old man developed fever, malaise, jaundice, pancytopenia, coagulation abnormalities, hepatomegaly, pleural effusions and a subcutaneous lump. Biopsies revealed a lobular panniculitis with the presence of cytophagic histiocytes; erythrophagocytosis was also demonstrated in the liver and bone marrow. Despite the use of chemotherapy (CHOP) his clinical condition deteriorated and he died 5 months after presentation. This illness is consistent with the recently described syndrome of histiocytic cytophagic panniculitis.

一名33岁男性出现发热、全身不适、黄疸、全血细胞减少、凝血异常、肝肿大、胸腔积液和皮下肿块。活检显示为小叶性脂膜炎，伴有噬血细胞组织细胞；肝脏和骨髓中也证实有红细胞吞噬现象。尽管使用了化疗（CHOP方案），但其临床状况仍恶化，就诊5个月后死亡。这种疾病符合最近描述的组织细胞性噬血细胞性脂膜炎综合征。