ABVD化疗后系统性皮肤指状突树突状细胞肉瘤完全缓解

Complete Remission in Systemic Skin Interdigitating Dendritic Cell Sarcoma after ABVD Chemotherapy.

作者信息

Kyogoku Chieko, Seki Masanori, Ogawa Shinichi, Miyamoto Kana, Ito Yufu, Kurita Naoki, Yokoyama Yasuhisa, Sakata-Yanagimoto Mamiko, Obara Naoshi, Hasegawa Yuichi, Fujishima Fumiyoshi, Ichinohasama Ryo, Nakamura Shigeo, Chiba Shigeru

机构信息

Department of Hematology, Faculty of Medicine, University of Tsukuba.

出版信息

J Clin Exp Hematop. 2015;55(1):33-7. doi: 10.3960/jslrt.55.33.

DOI:10.3960/jslrt.55.33

PMID:26106005

Abstract

Interdigitating dendritic cell sarcoma (IDCS) is a rare and aggressive neoplasm that is thought to arise from dendritic cells. This disease usually involves the lymph nodes and, rarely, extra-nodal sites. We report a 62-year-old man presenting skin nodules in the head, body, and extremities, as well as bone marrow involvement. Morphologic analysis of a biopsied specimen from the skin lesion was consistent with IDCS. Immunohistochemical staining demonstrated that the tumor cells were positive for IDCS-associated antigens such as CD4, CD45, CD68 (KP-1), and S-100 protein. Complete remission was achieved by treatment with 6 cycles of ABVD (adriamycin, bleomycin, vinblastine, and dacarbazine) chemotherapy. Although the optimal treatment of IDSC remains unknown, the experience in the current case supports the notion that ABVD chemotherapy may be effective for IDCS, and further extends this idea to rare patients presenting multiple skin lesions.

摘要

交错突细胞肉瘤（IDCS）是一种罕见的侵袭性肿瘤，被认为起源于树突状细胞。这种疾病通常累及淋巴结，很少累及结外部位。我们报告一名62岁男性，其头部、身体和四肢出现皮肤结节，并有骨髓受累。对皮肤病变活检标本的形态学分析与IDCS一致。免疫组织化学染色显示肿瘤细胞对IDCS相关抗原如CD4、CD45、CD68（KP-1）和S-100蛋白呈阳性。通过6个周期的ABVD（阿霉素、博来霉素、长春花碱和达卡巴嗪）化疗实现了完全缓解。尽管IDSC的最佳治疗方法仍不清楚，但本例的经验支持ABVD化疗可能对IDCS有效的观点，并将这一观点进一步扩展到出现多个皮肤病变的罕见患者。