Invernizzi R, Iannone A M, Bernuzzi S, D'Alessio A, Fiamenghi C, Fenoglio C, Rossi A, Bergamaschi G, Ascari E
Dipartimento di Medicina Interna, IRCCS Policlinico San Matteo, Pavia, Italy.
Leuk Lymphoma. 1995;18 Suppl 1:57-60. doi: 10.3109/10428199509075304.
In the hypergranular group of acute promyelocytic leukemia (APL) a rare subvariant with basophilic granules, metachromatic for toluidine blue, is recognizable. To evaluate the incidence as well as the biological and clinical significance of this subtype, we studied 53 consecutive untreated patients with APL with morphological, cytochemical, immunological and cytogenetic methods. In 10 cases (19% of the total) granules stained metachromatically in percentages of promyelocytes ranging from 16 to 60. In these cases peroxidase positivity was weaker than in the classic hypergranular and microgranular M3 and activities of esterases were usually present; at the ultrastructural level granules contained particulate material. Immunophenotypic and cytogenetic characteristics seemed not to differ from those of other M3 cases. Coagulopathy was usually life-threatening, notwithstanding the low white cell count, and the median survival was short. Hyperhistaminemia-related symptoms were not observed. Cytochemical, immunologic and cytogenetic findings are useful to differentiate this form from M2 with basophilic differentiation and from mast cell leukemia.
在急性早幼粒细胞白血病（APL）的粗颗粒组中，可识别出一种罕见的亚变体，其具有嗜碱性颗粒，对甲苯胺蓝呈异染性。为了评估该亚型的发生率以及生物学和临床意义，我们采用形态学、细胞化学、免疫学和细胞遗传学方法，对53例未经治疗的连续APL患者进行了研究。在10例患者（占总数的19%）中，早幼粒细胞中异染性染色的颗粒比例为16%至60%。在这些病例中，过氧化物酶阳性程度比经典的粗颗粒型和微颗粒型M3弱，酯酶活性通常存在；在超微结构水平上，颗粒含有颗粒状物质。免疫表型和细胞遗传学特征似乎与其他M3病例无异。尽管白细胞计数较低，但凝血障碍通常危及生命，中位生存期较短。未观察到与高组胺血症相关的症状。细胞化学、免疫学和细胞遗传学结果有助于将这种形式与具有嗜碱性分化的M2以及肥大细胞白血病区分开来。
