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一名患有纵隔生殖细胞肿瘤的患者出现伴有噬血细胞综合征的恶性组织细胞增生症/播散性组织细胞肉瘤。

Malignant histiocytoses/disseminated histiocytic sarcoma with hemophagocytic syndrome in a patient with mediastinal germ cell tumor.

作者信息

Shinoda Hiroko, Yoshida Akihiko, Teruya-Feldstein Julie

机构信息

Department of Pathology, Memorial Sloan-Kettering Cancer Center, New York, NY 10021, USA.

出版信息

Appl Immunohistochem Mol Morphol. 2009 Jul;17(4):338-44. doi: 10.1097/PAI.0b013e3181897e8e.

Abstract

We describe a case of a 24-year-old man with a large anterior mediastinal mass showing a nonseminomatous germ cell tumor then subsequently developed hemophagocytic syndrome involving the bone marrow and liver. During the course of chemotherapy, he developed profound thrombocytopenia, eccymoses, and bleeding. He had moderate splenomegaly and splenectomy was performed to restore adequate hematologic reserve to permit further chemotherapy. The spleen showed marked erythrophagocytosis and markedly atypical histiocytes consistent with malignant histiocytoses. Atypical histiocytes stained positive for CD68, CD163, CD4, CD45 (LCA), and S-100. Cytogenetics studies were negative for i12p. The patient was refractory to therapy and ultimately died 5 months after diagnosis.

摘要

我们描述了一例24岁男性患者,其前纵隔有一个大肿块,病理显示为非精原细胞瘤性生殖细胞肿瘤,随后发展为累及骨髓和肝脏的噬血细胞综合征。在化疗过程中,他出现了严重的血小板减少、瘀斑和出血。他有中度脾肿大,遂行脾切除术以恢复足够的血液学储备,以便进一步化疗。脾脏显示有明显的红细胞吞噬现象以及与恶性组织细胞增多症一致的明显非典型组织细胞。非典型组织细胞CD68、CD163、CD4、CD45(白细胞共同抗原)和S-100染色呈阳性。细胞遗传学研究显示i12p为阴性。该患者对治疗无效,最终在诊断后5个月死亡。

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