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原发性纵隔非精原细胞瘤性生殖细胞肿瘤伴噬血细胞综合征。

Primary mediastinal non-seminomatous germ cell tumor associated with hemophagocytic syndrome.

作者信息

Sada Eriko, Shiratsuchi Motoaki, Kiyasu Junnichi, Idutsu Kensaku, Ohtsuka Rie, Nagasawa Eriko, Karube Kennosuke, Takayanagi Ryoichi, Abe Yasunobu

机构信息

Department of Medicine and Bioregulatory Science, Graduate School of Medical Sciences, Kyushu University, Higashi-ku, Fukuoka, Japan.

出版信息

J Clin Exp Hematop. 2009 Nov;49(2):117-20. doi: 10.3960/jslrt.49.117.

Abstract

A 20-year-old man with a primary non-seminomatous mediastinal germ cell tumor (yolk sac tumor and immature teratoma) developed hemophagocytic syndrome (HPS) three months after surgical resection. Around the same time, the patient was found to have bone metastases of the germ cell tumor. No other hereditary or acquired diseases related to HPS were found. The thrombocytopenia was refractory to corticosteroid therapy but improved after chemotherapy performed for germ cell tumor progression. Only three cases of germ cell tumor associated with reactive hemophagocytosis have been previously reported. Successful treatment of the present case by chemotherapy for HPS suggests a close relationship between this rare complication and germ cell tumor.

摘要

一名20岁男性患有原发性非精原性纵隔生殖细胞肿瘤(卵黄囊瘤和未成熟畸胎瘤),手术切除三个月后发生噬血细胞综合征(HPS)。大约在同一时间,该患者被发现患有生殖细胞肿瘤骨转移。未发现其他与HPS相关的遗传性或获得性疾病。血小板减少症对皮质类固醇治疗无效,但在针对生殖细胞肿瘤进展进行化疗后有所改善。此前仅报道过三例与反应性噬血细胞增多相关的生殖细胞肿瘤病例。本病例通过化疗成功治疗HPS表明这种罕见并发症与生殖细胞肿瘤之间存在密切关系。

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