Kass L, Hadi M Z
Am J Clin Pathol. 1975 Oct;64(4):503-8. doi: 10.1093/ajcp/64.4.503.
Phosphorylase activity was detected in the cytoplasm of erythroid precursors of 6 of 7 patients with chronic erythremic myelosis (Di Guglielmo syndrome), in proerythroblasts and megaloblasts from 3 patients with pernicious anemia and in 2 patients with severe folate deficiency in neoplastic lymphocytes from 2 patients with acute lymphoblastic leukemia, and in 1 patient with leukemic lymphosarcoma. In all of these patients, most of the erythroid precursors and/or neoplastic lymphocytes contained increased amounts of glycogen when stained with the PAS reagent. Phosphorylase activity was not detected in erythroid precursors obtained from 6 presumed normal individuals or from 3 of 7 patients with a variety of other types of anemia in which the erythroid precursors were PAS-negative. Similarly, phosphorylase activity was absent in lymphocytes obtained from presumed normal individuals. Although the mechanisms responsible for the pathogenesis of PAS positivity are unclear, it is possible that the increased phosphorylase activity found in cells that are PAS-positive may reflect a disorder in the biosynthetic pathway of glycogen.
在7例慢性红细胞增多性骨髓增生症（迪古列尔莫综合征）患者中的6例、3例恶性贫血患者的早幼红细胞和巨幼红细胞、2例严重叶酸缺乏患者的急性淋巴细胞白血病的肿瘤性淋巴细胞以及1例白血病性淋巴肉瘤患者中，均检测到磷酸化酶活性。在所有这些患者中，当用PAS试剂染色时，大多数红细胞前体和/或肿瘤性淋巴细胞含有增加量的糖原。在6名推测为正常个体或7例其他各种类型贫血患者中的3例（这些患者的红细胞前体PAS染色为阴性）所获得的红细胞前体中，未检测到磷酸化酶活性。同样，在推测为正常个体的淋巴细胞中也不存在磷酸化酶活性。尽管导致PAS阳性发病机制的原因尚不清楚，但在PAS阳性细胞中发现的磷酸化酶活性增加可能反映了糖原生物合成途径的紊乱。
