Najfeld V, Zucker-Franklin D, Adamson J, Singer J, Troy K, Fialkow P J
Polly Annenberg Levee Hematology Center, Mount Sinai Medical Center, New York, New York 10029.
Leukemia. 1988 Jun;2(6):351-7.
Previous studies have shown that acute nonlymphocytic leukemias are clonal diseases in which there is heterogeneity in the pattern of stem cell differentiative expression. To determine whether M7 megakaryocytic leukemia is a clonal disease and to evaluate the differentiative expression of the cells involved by the leukemia we studied a patient with megakaryocytic leukemia who was heterozygous for the X-chromosome-linked glucose-6-phosphate dehydrogenase (G6PD). The diagnosis of megakaryocytic leukemia was based on results obtained with the immunogold method and ultrastructural studies with the monoclonal anti-Gplla/IIIb antibody, 10E5. Direct testing of blood and marrow mononuclear cells and blood platelets demonstrated only A-type G6PD, whereas skin exhibited both B and A enzymes. The results indicate that the megakaryocytic leukemia in this patient was clonal at the time of study. To determine the differentiative expression of the stem cells, granulocyte/macrophage colony forming units and erythroid burst forming units were cultured and the resultant colonies were tested for G6PD. The results indicate that the stem cells involved by the leukemia exhibited differentiative expression multipotent for the megakaryocytic and granulocytic pathways, but no definitive conclusion could be made regarding the erythroid lineage.
以往的研究表明，急性非淋巴细胞白血病是克隆性疾病，其中干细胞分化表达模式存在异质性。为了确定M7巨核细胞白血病是否为克隆性疾病，并评估白血病所涉及细胞的分化表达，我们研究了一名患有巨核细胞白血病的患者，该患者为X染色体连锁葡萄糖-6-磷酸脱氢酶（G6PD）杂合子。巨核细胞白血病的诊断基于免疫金法和使用单克隆抗Gplla/IIIb抗体10E5的超微结构研究结果。对血液和骨髓单核细胞以及血小板的直接检测仅显示A型G6PD，而皮肤则同时显示B型和A型酶。结果表明，该患者的巨核细胞白血病在研究时是克隆性的。为了确定干细胞的分化表达，培养了粒细胞/巨噬细胞集落形成单位和红系爆式集落形成单位，并对所得集落进行了G6PD检测。结果表明，白血病所涉及的干细胞在巨核细胞和粒细胞途径中表现出多能分化表达，但对于红系谱系无法得出明确结论。
