Shimizu Yoko, Tanae Ken, Takahashi Naoki, Kohri Mika, Arai Eiichi, Bessho Masami, Niitsu Nozomi
Department of Hematology, Comprehensive Cancer Center, International Medical Center, Saitama Medical University, 1397-1 Yamane, Hidaka, Saitama 350-1298, Japan.
Leuk Res. 2010 Feb;34(2):263-6. doi: 10.1016/j.leukres.2009.07.001. Epub 2009 Jul 28.
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.
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。