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伴噬血细胞综合征的原发性皮肤间变性大细胞淋巴瘤:1例报告并文献复习

Primary cutaneous anaplastic large-cell lymphoma presenting with hemophagocytic syndrome: a case report and review of the literature.

作者信息

Shimizu Yoko, Tanae Ken, Takahashi Naoki, Kohri Mika, Arai Eiichi, Bessho Masami, Niitsu Nozomi

机构信息

Department of Hematology, Comprehensive Cancer Center, International Medical Center, Saitama Medical University, 1397-1 Yamane, Hidaka, Saitama 350-1298, Japan.

出版信息

Leuk Res. 2010 Feb;34(2):263-6. doi: 10.1016/j.leukres.2009.07.001. Epub 2009 Jul 28.

Abstract

Primary cutaneous anaplastic large-cell lymphoma (C-ALCL) is a rare entity of lymphoma. We report a case of C-ALCL presenting with hemophagocytic syndrome and skin lesion with giant ulcer. Histopathological examination of the skin biopsy specimens showed non-epidermotropic infiltrates with cohesive sheets of large tumor cells. The tumor cells showed CD4-, CD8+, CD30+, CD56-, ALK-, TIA-1+, and granzyme B+. C-ALCL is generally a disorder that progresses slowly and has a good prognosis. Manifestation of a giant ulcer and hemophagocytic syndrome, such as in the present case, is rare.

摘要

原发性皮肤间变性大细胞淋巴瘤(C-ALCL)是一种罕见的淋巴瘤类型。我们报告一例C-ALCL,伴有噬血细胞综合征及巨大溃疡的皮肤病变。皮肤活检标本的组织病理学检查显示非亲表皮性浸润,有大片紧密排列的大肿瘤细胞。肿瘤细胞表现为CD4阴性、CD8阳性、CD30阳性、CD56阴性、ALK阴性、TIA-1阳性及颗粒酶B阳性。C-ALCL通常是一种进展缓慢且预后良好的疾病。如本例所示,出现巨大溃疡和噬血细胞综合征的表现较为罕见。

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