急性粒-单核细胞白血病合并指状突树突细胞肉瘤一例并文献复习

One case of interdigitating dendritic cell sarcoma concurrent with acute myelomonocytic leukemia and literatures review

目的进一步认识指状突树突细胞肉瘤（IDCS）的生物学属性和临床特征。方法以迄今所报道的第一例IDCS合并急性粒-单核细胞白血病患者为研究对象，结合文献复习总结该病的生物学及临床特征。结果该例患者有前驱肿瘤病史及放化疗史，考虑为治疗相关性树突细胞肉瘤，根据实验室检测结果考虑两种肿瘤同为髓系来源。患者对连续4次化疗反应不佳。数据库检索到的62例IDCS患者中，17％曾有恶性肿瘤及放化疗治疗史，此类患者预后更为恶劣。结论IDCS预后差，其中治疗相关性类型预后更差。加强对接受化疗和（或）放疗的肿瘤或非肿瘤患者的癌症预防和早期检测有重要的临床意义。

Objective To summarize and learn the biological properties and clinical features of interdigitating dendritic cell sarcomas （IDCS）. Methods The first IDCS patient concurrent with acute myelomonocytic leukemia （AML-M4） described herein, to our knowledge, was studied and 62 IDCS cases reported previously in the literature were reviewed. Results The patient had a history of breast cancer as well as radiotherapy and chemotherapy of it, and the patient showed poor response to 4 cycles of sequential ehemocherapy regimens. Based on the laboratory results, IDCS and AML-M4 in this patient were both of myelogenous origination. Furthermore, review of the 62 IDCS patients reported previously showed that as high as 17 % of the patients had malignant disease and received radiotherapy or chemotherapy before they got IDCS, and patients of this group had worse prognosis compared with counterpart. Conclusion IDCS has poor prognosis, and therapy-related type worse. Prophylactic measures and stringent screening of the second cancer in those who received chemoterapy or radiotherapy are appropriated and necessary.