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急性早幼粒细胞白血病:39例研究及一种嗜碱性过强微颗粒变异型的鉴定

Acute promyelocytic leukaemia: a study of 39 cases with identification of a hyperbasophilic microgranular variant.

作者信息

McKenna R W, Parkin J, Bloomfield C D, Sundberg R D, Brunning R D

出版信息

Br J Haematol. 1982 Feb;50(2):201-14. doi: 10.1111/j.1365-2141.1982.tb01910.x.

DOI:10.1111/j.1365-2141.1982.tb01910.x
PMID:6949609
Abstract

Thirty-nine cases of acute promyelocytic leukaemia (APL) were divided into two morphological subgroups, typical hypergranular APL (31 cases) and microgranular APL (eight cases, 21%). The leukaemic cells in the microgranular APL cases were characterized by striking nuclear folding or lobulation; granulation was present in most of these cells but was less abundant and finer than in typical APL. In three microgranular APL cases a distinctive small leukaemic promyelocyte with unusual nuclear lobulation and deeply basophilic cytoplasm containing few or no visable granules was the predominant leukaemic cell. This small hyperbasophilic promyelocyte was also present as a minor population of cells in the other five microgranular APL case and in 28 of the 31 typical APL cases. Ultrastructurally the most abundant promyelocytes in microgranular APL had smaller and usually fewer granules than in typical APL; other characteristic ultrastructural features of APL were found with equal frequency. The median blood leucocyte count was significantly higher in microgranular APL, 83.0 x 10(9) x 10(9)/l, than in typical APL, 1.8 x 10(9)/l (P less than 0.01). The median duration of complete remission (CR) for microgranular APL, 6.5 months, was shorter than the 21 + month median CR for typical APL. The morphological characteristics of microgranular APL may mimic those of myelomonocytic leukaemia; however, the presence of cells with multiple Auer rods, large inclusions of Auer-like material and the small hyperbasophilic promyelocytes are important distinguishing features. In equivocal cases cytochemistry, electron microscopy and cytogenetic studies may verify the diagnosis.

摘要

39例急性早幼粒细胞白血病(APL)被分为两个形态学亚组,典型的颗粒增多型APL(31例)和微颗粒型APL(8例,占21%)。微颗粒型APL病例中的白血病细胞特征为显著的核折叠或分叶;这些细胞大多有颗粒,但比典型APL中的颗粒数量少且更细小。在3例微颗粒型APL病例中,一种独特的小白血病早幼粒细胞是主要的白血病细胞,其核分叶异常,胞质深嗜碱性,含很少或无可见颗粒。这种小的嗜碱性增强的早幼粒细胞在其他5例微颗粒型APL病例以及31例典型APL病例中的28例中也作为少数细胞群体存在。超微结构上,微颗粒型APL中最丰富的早幼粒细胞比典型APL中的颗粒更小且通常更少;APL的其他特征性超微结构特征出现频率相同。微颗粒型APL的中位血白细胞计数显著高于典型APL,分别为83.0×10⁹/L和1.8×10⁹/L(P<0.01)。微颗粒型APL的完全缓解(CR)中位持续时间为6.5个月,短于典型APL的21 +个月的CR中位持续时间。微颗粒型APL的形态学特征可能类似于粒单核细胞白血病;然而,存在具有多条Auer小体、大量Auer样物质包涵体以及小的嗜碱性增强的早幼粒细胞是重要的鉴别特征。在疑难病例中,细胞化学、电子显微镜检查和细胞遗传学研究可证实诊断。

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