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佩吉特样网状细胞增生症(沃林格-科洛普病)。一项超微结构和免疫细胞学研究。

Pagetoid reticulosis (Woringer-Kolopp disease). An ultrastructural and immunocytological study.

作者信息

Takahashi H, Takahashi K, Tanno K, Iijima S

出版信息

Acta Pathol Jpn. 1982 May;32(3):513-26. doi: 10.1111/j.1440-1827.1982.tb01407.x.

DOI:10.1111/j.1440-1827.1982.tb01407.x
PMID:6980557
Abstract

Histopathological, immunocytological and ultrastructural observations are reported in the first case of pagetoid reticulosis (Woringer-Kolopp disease) in Japan. The patient was a 61-year-old woman with multiple skin lesions running a chronic and apparently benign clinical course. Histology of the skin biopsies revealed typical pagetoid appearance of the epidermis due to intraepidermal infiltration of abnormal cells. Ultrastructural investigation showed that the intraepidermal abnormal cells were classified into mycosis fungoides cells, Sézary cells, lymphoblast-like cells, and large blastoid cells and that the mycosis fungoides cells were a major cell population. Intermediate or transitional cells were found between these cells and large blastoid cells were mostly situated in the basal cell layer. By the rosetting assays of the free cell suspensions prepared from the epidermis of the biopsied skin lesions, 93% of the suspended cells were positive for spontaneous rosette formation with sheep erythrocytes. The immunoperoxidase technique demonstrated no cytoplasmic immunoglobulins in almost all the intraepidermal abnormal cells. These results indicate that the intraepidermal abnormal cells are T-lymphocytes. Thus, it is concluded that the present case is a cutaneous T-cell lymphoma of low-grade malignancy showing a prominent epidermotropism. This case is the first description of the disease in Japan.

摘要

本文报道了日本首例派杰样网状细胞增生症(沃林格-科洛普病)的组织病理学、免疫细胞学及超微结构观察结果。患者为一名61岁女性,有多处皮肤损害,病程呈慢性且显然为良性。皮肤活检组织学检查显示,由于异常细胞在表皮内浸润,表皮呈现典型的派杰样外观。超微结构研究表明,表皮内异常细胞可分为蕈样霉菌病细胞、赛塞里细胞、淋巴母细胞样细胞和大母细胞样细胞,其中蕈样霉菌病细胞是主要细胞群体。在这些细胞之间发现了中间或过渡细胞,大母细胞样细胞大多位于基底细胞层。通过对取自活检皮肤损害表皮的游离细胞悬液进行玫瑰花结试验,93%的悬浮细胞与绵羊红细胞自发形成玫瑰花结呈阳性。免疫过氧化物酶技术显示,几乎所有表皮内异常细胞均无细胞质免疫球蛋白。这些结果表明,表皮内异常细胞为T淋巴细胞。因此,得出结论,本例为低度恶性的皮肤T细胞淋巴瘤,表现出明显的亲表皮性。这是日本对该疾病的首次描述。

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