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骨髓和脾脏中毛细胞白血病的诊断与鉴别诊断

The diagnosis and differential diagnosis of hairy cell leukemia in bone marrow and spleen.

作者信息

Burke J S, Rappaport H

出版信息

Semin Oncol. 1984 Dec;11(4):334-46.

PMID:6505703
Abstract

In this study we reviewed the morphologic features of marrow biopsies and aspirates as well as splenic sections derived from 28 patients with hairy cell leukemia. Marrow biopsies proved reliable in establishing and/or confirming the diagnosis in every patient, when available for review. Regardless of the degree of marrow involvement, the hairy cell infiltrates consistently exhibited wide spacing of their nuclei due to relatively abundant pale to clear cytoplasm. Hairy cells appeared homogeneous and bland, without mitotic activity or prominent nucleoli; nuclear contours characteristically were ovoid and to a lesser extent slightly indented or reniform. The splenic histology of hairy cell leukemia was equally distinctive. The splenic red pulp was diffusely infiltrated by a uniform population of cytologically monotonous mononuclear cells that expanded the red pulp cords, filled the sinuses, and generally led to atrophy or obliteration of the white pulp. Moreover, the clear cytoplasm of hairy cells usually was highlighted in sinusoidal blood-filled lakes. Despite the employment of these characteristic morphologic criteria for the diagnosis of hairy cell leukemia in marrow and spleen, these pathologic changes may vary and may be simulated in part by a variety of other hematologic disorders. Accuracy of diagnosis requires not only knowledge of the usual pathologic features of hairy cell leukemia but also knowledge of the unusual. Awareness of these pathologic variations will aid in the improvement of diagnosis and will provide a foundation for understanding the clinical and biologic aspects of hairy cell leukemia.

摘要

在本研究中,我们回顾了28例毛细胞白血病患者的骨髓活检、骨髓穿刺及脾切片的形态学特征。当可用于检查时,骨髓活检在每位患者的诊断确立和/或确认方面均被证明是可靠的。无论骨髓受累程度如何,毛细胞浸润始终表现为细胞核间距较宽,这是由于细胞质相对丰富且淡染至清亮。毛细胞外观均一且平淡,无有丝分裂活性或明显核仁;核轮廓典型地呈卵圆形,在较小程度上略有凹陷或呈肾形。毛细胞白血病的脾脏组织学同样具有特征性。脾红髓被一群形态学单一的单核细胞弥漫浸润,这些细胞使红髓索增宽,充满血窦,通常导致白髓萎缩或消失。此外,在充满血液的窦状隙中,毛细胞清亮的细胞质通常很突出。尽管采用了这些骨髓和脾脏毛细胞白血病诊断的特征性形态学标准,但这些病理变化可能有所不同,并且可能部分被多种其他血液系统疾病所模拟。准确的诊断不仅需要了解毛细胞白血病的常见病理特征,还需要了解其不常见特征。认识到这些病理变化将有助于提高诊断水平,并为理解毛细胞白血病临床和生物学方面提供基础。

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