Effective high-dose chemotherapy followed by autologous peripheral blood stem cell transplantation in a patient with the aggressive form of cytophagic histiocytic panniculitis
Effective high-dose chemotherapy followed by autologous peripheral blood stem cell transplantation in a patient with the aggressive form of cytophagic histiocytic panniculitis
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DOI：
10.1038/sj.bmt.1700858
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发表时间：
1997-07-01
影响因子：
4.8
通讯作者：
Koike, T
中科院分区：
文献类型：
--
作者：
Koizumi, K;Sawada, K;Koike, T
1例20岁日本男性患者出现全身皮下无痛结节、发热、肝功能异常、浆膜积液、肝脾肿大、淋巴结肿大和贫血，皮肤活检发现小叶性脂膜炎，形态上为良性组织细胞浸润和明显的吞噬。皮肤和肝脏也有不典型的T淋巴细胞，诊断为侵袭性细胞吞噬组织细胞性脂膜炎(CHP)或侵袭性皮下脂膜下T细胞淋巴瘤(SPTCL)，第1天接受环磷酰胺、阿霉素和长春新碱治疗，第1-5天接受强的松龙治疗，第1、3、5天接受依托泊苷(CHOP-E)治疗，在粒细胞集落刺激因子的支持下，每2周重复一次，经过3个CHOP-E周期后临床完全缓解(CCR)。由于侵袭性CHP的临床病程反复且往往是致命的，他接受了大剂量化疗和自体外周血干细胞移植(APBSCT)，经过5个周期的CHOP-E，APBSCT后他已经在CCR住了12个月，高剂量化疗然后APBSCT被认为是对侵袭性CHP和侵袭期SPTCL患者最有益的治疗方法之一。
A 20-year-old Japanese man developed generalized, subcutaneous, painless nodules, fever, abnormal liver function, serosal effusions, hepatosplenomegaly, lymphadenopathy and anemia, Skin biopsies revealed lobular panniculitis with a morphologically benign histiocytic infiltration and prominent phagocytosis. Atypical T lymphocytes were also present in the skin and liver, The diagnosis given was aggressive cytophagic histiocytic panniculitis (CHP) or aggressive subcutaneous panniculitic T cell lymphoma (SPTCL), He received cyclophosphamide, doxorubicin, and vincristine on day 1, prednisolone on days 1-5, and etoposide on days 1, 3 and 5 (CHOP-E), with the support of granulocyte colony-stimulating factor, This regimen was repeated every 2 weeks and complete clinical remission (CCR) was attained after three cycles of CHOP-E. As the clinical course of aggressive CHP is recurrent and often fatal, he was given high-dose chemotherapy followed by autologous peripheral blood stem cell transplantation (APBSCT), after five cycles of CHOP-E, He has remained in CCR for 12 months after APBSCT, High-dose chemotherapy followed by APBSCT is considered to be one of the most beneficial therapies for patients with aggressive CHP and aggressive phase SPTCL.