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脾假血窦和肝血管瘤样病变。毛细胞白血病的特征。

Splenic pseudosinuses and hepatic angiomatous lesions. Distinctive features of hairy cell leukemia.

作者信息

Nanba K, Soban E J, Bowling M C, Berard C W

出版信息

Am J Clin Pathol. 1977 May;67(5):415-26. doi: 10.1093/ajcp/67.5.415.

Abstract

In 14 cases of hairy cell leukemia unique vascular lesions not previously reported were observed in surgically resected spleens and hepatic biopsies. In all 14 spleens there were variably prominent distended spaces filled with erythrocytes and resembling dilated sinuses. These structures appeared to be lined by hairy cells,and studies utilizing histochemical and enzymatic methods confirmed that they were in fact pseudosinuses lacking the endothelial cells and ring fibers of normal splenic sinuses. These changes were not present in normal spleens or in spleens involved by other types of chronic leukemia, and appear to be qualitatively specific for hairy cell leukemia. In three of five hepatic biopsies similar angiomatous lesions were present in multifocal clusters resembling hemangiomas. Their pathogenesis may be related to possible adherence of hairy cells to reticulum fibers in involved tissues.

摘要

在14例毛细胞白血病患者中,通过手术切除脾脏和肝脏活检发现了以前未报道过的独特血管病变。在所有14个脾脏中,均可见不同程度突出的扩张间隙,内充满红细胞,类似扩张的血窦。这些结构似乎由毛细胞排列而成,利用组织化学和酶学方法进行的研究证实,它们实际上是假血窦,缺乏正常脾血窦的内皮细胞和环状纤维。这些变化在正常脾脏或其他类型慢性白血病累及的脾脏中不存在,似乎在性质上是毛细胞白血病所特有的。在5例肝脏活检中,有3例存在类似血管瘤的多灶性簇状血管病变。其发病机制可能与毛细胞在受累组织中与网状纤维的可能黏附有关。

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