Matsue K, Itoh M, Tsukuda K, Miyazaki K, Kokubo T
Department of Medicine, Kameda General Hospital, Chiba, Japan.
Am J Clin Oncol. 1994 Dec;17(6):470-4. doi: 10.1097/00000421-199412000-00003.
A 27-year-old woman developed generalized subcutaneous painful nodules, fever, abnormal liver function, a bleeding tendency, and pancytopenia. Skin biopsies revealed the lobular panniculitis with a morphologically benign histiocytic infiltration with prominent phagocytosis. Leukophagocytosis and erythrophagocytosis were also present in the bone marrow. The diagnosis of cytophagic histiocytic panniculitis was made. The patient received polychemotherapy with cyclophosphamide, Adriamycin, and vincristine on day 1, prednisone on day 1-5 (modified CHOP), with the addition of etoposide (E). This regimen was repeated 8 times every 3 weeks. The patient obtained a complete clinical remission that has lasted almost 2 years after the completion of chemotherapy. Thus we suggest modified CHOP-E chemotherapy for an effective treatment for the aggressive form of cytophagic histiocytic panniculitis.
一名27岁女性出现全身性皮下疼痛性结节、发热、肝功能异常、出血倾向和全血细胞减少。皮肤活检显示小叶性脂膜炎，伴有形态学上良性的组织细胞浸润及显著的吞噬作用。骨髓中也存在噬白细胞现象和噬红细胞现象。诊断为噬血细胞性组织细胞性脂膜炎。患者于第1天接受环磷酰胺、阿霉素和长春新碱的联合化疗，第1 - 5天接受泼尼松（改良CHOP方案），并加用依托泊苷（E）。该方案每3周重复1次，共进行8次。患者在化疗结束后获得了持续近2年的完全临床缓解。因此，我们建议采用改良CHOP - E化疗方案有效治疗侵袭性噬血细胞性组织细胞性脂膜炎。
