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成人T细胞白血病/淋巴瘤(ATLL)在皮肤中的血管中心性和血管破坏性浸润。两例报告。

Angiocentric and angiodestructive infiltration of adult T-cell leukemia/lymphoma (ATLL) in the skin. Report of two cases.

作者信息

Manabe T, Hirokawa M, Sugihara K, Sugihara T, Kohda M

机构信息

Department of Pathology, Kawasaki Medical School, Kurashiki, Japan.

出版信息

Am J Dermatopathol. 1988 Dec;10(6):487-96. doi: 10.1097/00000372-198812000-00003.

Abstract

This report describes the clinical and pathological features of cutaneous lesions observed in a 73-year-old woman with terminal adult T-cell leukemia/lymphoma (ATLL) and a 42-year-old man who had chronic ATLL with persistent nasal stuffiness and rhinorrhea of 2 years' duration. Histology of the skin lesions from these two cases showed large blood vessel (arterial and venous) involvement at the level of the dermosubcutaneous junction, in which angiocentric and angiodestructive infiltration of the tumor cells was prominent and reminiscent of lymphomatoid granulomatosis (LYG) and/or polymorphic reticulosis. ATLL commonly involves the skin. Moreover, it may originate in the skin. The majority of researchers have observed perivascular and periadnexal infiltration, frequent epidermal exocytosis with Pautrier's microabscesses, and the occasional presence of leukemic cells within the vascular lumen; however, vascular involvement like that seen in our cases has not been described before. Our cases suggest that a variety of T-cell malignancies may be angiocentric and angiodestructive in nature, and that so-called LYG and LYG-like malignant lymphomas are not a single disease, but may constitute a heterogeneous group of T-cell malignancies including ATLL.

摘要

本报告描述了一名73岁终末期成人T细胞白血病/淋巴瘤(ATLL)女性患者以及一名42岁患有慢性ATLL且持续鼻塞和流涕2年的男性患者皮肤病变的临床和病理特征。这两例患者皮肤病变的组织学检查显示,在真皮皮下交界处存在大血管(动脉和静脉)受累,肿瘤细胞呈血管中心性和血管破坏性浸润,这与淋巴瘤样肉芽肿病(LYG)和/或多形性网状细胞增多症相似。ATLL常累及皮肤。此外,它可能起源于皮肤。大多数研究人员观察到血管周围和腺周浸润、伴有Pautrier微脓肿的频繁表皮内渗以及血管腔内偶尔出现白血病细胞;然而,像我们病例中所见的血管受累情况此前尚未见报道。我们的病例表明,多种T细胞恶性肿瘤在本质上可能是血管中心性和血管破坏性的,并且所谓的LYG和LYG样恶性淋巴瘤并非单一疾病,而是可能构成包括ATLL在内的一组异质性T细胞恶性肿瘤。

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