De Salvo Cardullo L, Weir Madina J, Gómez Sánchez O, Plumacher Z, Salas D
Instituto Hematológico de Occidente-Banco de Sangre del Estado Zulia, Maracaibo, Venezuela.
Invest Clin. 1990;31(3):153-61.
One of the main criteria in the differentiation between acute lymphoblastic (ALL) and acute myeloblastic leukemias (AML) is the presence of granules in the blasts of the latter. Recently, several groups have described a form of ALL with prominent intracytoplasmatic granules (G-ALL) in the blasts. The granules in the G-ALL blasts do not contain myeloperoxidase, but sometimes have lipids that stain with Sudan black B (SBB). We describe a case of G-ALL in a five-year-old girl whose peripheral blood and bone marrow was compound of 98% lymphoblasts, 30% of which, had prominent azurophilic intracytoplasmatic granules. The granules did not have peroxidase, acid phosphatase, varies; is directly proportional to naphthyl acetate esterase. However 5% of the blasts had sudanophilic granules and 60% were positive for the periodic acid-Schiff reaction. The blasts expressed the CD10 (CALLA) and Dr antigens, and were negative for surface immunoglobulins or the CD4, CD8, or CD14, antigens. Only 18% of cells formed rosettes with sheep erythrocytes. The patient responded to vincristine, prednisone and L-asparaginase. Based on the finding we diagnosed this as a CALLA positive G-ALL. By conventional criteria this case would have been wrongly classified as AML.
急性淋巴细胞白血病(ALL)与急性髓细胞白血病(AML)鉴别的主要标准之一是后者的原始细胞中存在颗粒。最近,有几个研究小组描述了一种原始细胞中有显著胞质内颗粒的ALL形式(颗粒型ALL,G-ALL)。G-ALL原始细胞中的颗粒不含髓过氧化物酶,但有时含有能被苏丹黑B(SBB)染色的脂质。我们报告一例5岁女孩的G-ALL病例,其外周血和骨髓中98%为原始淋巴细胞,其中30%有显著的嗜天青颗粒。这些颗粒没有过氧化物酶、酸性磷酸酶,酯酶;与醋酸萘酯酶成正比。然而,5%的原始细胞有嗜苏丹颗粒,60%对过碘酸-希夫反应呈阳性。原始细胞表达CD10(普通急性淋巴细胞白血病抗原,CALLA)和Dr抗原,表面免疫球蛋白或CD4、CD8或CD14抗原呈阴性。只有18%的细胞与绵羊红细胞形成玫瑰花结。该患者对长春新碱、泼尼松和L-天冬酰胺酶有反应。基于这些发现,我们将其诊断为CALLA阳性G-ALL。按照传统标准,该病例会被错误地归类为AML。