Chan W C, Brynes R K, Kim T H, Verras A, Schick C, Green R J, Ragab A H
Blood. 1983 Jul;62(1):92-8.
Two girls, each less than 2 yr of age, developed acute megakaryoblastic leukemia (malignant myelosclerosis). Both presented with anemia, severe thrombocytopenia, and a low percentage of blasts in their peripheral blood. Their marrow showed marked reticulin fibrosis with an increase in blasts and immature megakaryocytes. The blasts stained negatively for myeloperoxidase and Sudan Black B, but showed acid phosphatase (ACP) and alpha-naphthyl acetate esterase (ANAE) activity inhibitable by sodium fluoride. They were identified as megakaryoblasts by the platelet peroxidase reaction. Cytogenetic studies showed multiple chromosomal abnormalities in both cases. Chemotherapy with vincristine, prednisone, and L-asparaginase was without effect, while daunorubicin and cytosine arabinoside induced a complete remission in one case. The second case responded to a combination of cytosine arabinoside, daunorubicin, and 6-thioguanine. This article documents that acute megakaryoblastic leukemia occurs in early childhood and describes its clinical, pathologic, and cytogenetic features. Previous reports of childhood "myelofibrosis" are reviewed, and their possible relationship with acute megakaryoblastic leukemia is discussed.
两名年龄均小于2岁的女童患上了急性巨核细胞白血病（恶性骨髓硬化症）。两人均表现为贫血、严重血小板减少以及外周血中原始细胞比例较低。她们的骨髓显示出明显的网状纤维组织增生，伴有原始细胞和未成熟巨核细胞增多。原始细胞髓过氧化物酶和苏丹黑B染色均为阴性，但显示酸性磷酸酶（ACP）和α-萘乙酸酯酶（ANAE）活性可被氟化钠抑制。通过血小板过氧化物酶反应，它们被鉴定为巨核母细胞。细胞遗传学研究显示，两例均存在多种染色体异常。使用长春新碱、泼尼松和L-天冬酰胺酶进行化疗无效，而柔红霉素和阿糖胞苷在一例中诱导了完全缓解。第二例对阿糖胞苷、柔红霉素和6-硫鸟嘌呤的联合治疗有反应。本文记录了急性巨核细胞白血病在幼儿期发病，并描述了其临床、病理和细胞遗传学特征。对先前关于儿童“骨髓纤维化”的报道进行了回顾，并讨论了它们与急性巨核细胞白血病的可能关系。
