Cerveró C, Heinrichs B, Villarrubia J, Velasco J L, Ferro M T, López J, Escribano L
Servicio de Hematología, Hospital Ramón y Cajal, Madrid.
Sangre (Barc). 1994 Apr;39(2):135-8.
The pseudo-Chédiak-Higashi anomaly is characterized by the presence of giant granules in the cytoplasm of blast cells in acute leukemia. We report here a new case of acute myelogenous leukemia (M2 type) with this alteration. The granules were azurophilic or eosinophilic and reacted strongly to peroxidase stain. Ultrastructural studies showed that the granules contained a dense matrix and occasionally "finger print" structures at the periphery; in some inclusions, fibrillar structures of myelinic figures could be seen. The matrix was reactive to peroxidase and small vesicles were prominent in the cytoplasm near the granules. We conclude that the giant granules could have been formed by the fusion of primary granules and/or by the fusion of these small dense vesicles.
假性切-东综合征的特征是急性白血病原始细胞胞质中出现巨大颗粒。我们在此报告1例具有这种改变的急性髓系白血病(M2型)新病例。这些颗粒呈嗜天青或嗜酸性,对过氧化物酶染色反应强烈。超微结构研究显示,这些颗粒含有致密基质,外周偶尔可见“指纹”结构;在一些包涵体中,可见髓鞘样结构的纤维状结构。基质对过氧化物酶有反应,颗粒附近的细胞质中可见小泡。我们得出结论,巨大颗粒可能是由初级颗粒融合和/或这些小的致密小泡融合形成的。