Travis W D, Li C Y, Banks P M, Nichols W L
Cancer. 1987 Jul 15;60(2):193-200. doi: 10.1002/1097-0142(19870715)60:2<193::aid-cncr2820600213>3.0.co;2-u.
Three cases of megakaryoblastic transformation of chronic granulocytic leukemia (CGL) are reported. In each case, the leukemic transformation had morphologic features suggesting megakaryocytic differentiation. This was confirmed by positive immunostaining with a monoclonal antibody (HP1-1D) specific for platelet and megakaryocyte glycoprotein IIb/IIIa antigen, which was expressed by the majority of the leukemic blasts in all three cases. Cases with evidence of multilineage differentiation of the leukemic transformation were excluded. A striking feature in two patients was the manifestation of lytic bone lesions and soft tissue masses at presentation. A biopsy of a lytic bone lesion and soft tissue mass in one patient revealed a megakaryoblastic leukemic infiltrate, which by immunocytochemical staining was positive for the megakaryocytic markers, glycoprotein IIb/IIIa antigen, and Factor VIII (von Willebrand factor) antigen. In contrast to granulocytic sarcomas, the megakaryoblastic sarcoma did not stain cytochemically for chloroacetate esterase. The mean survival after acute transformation was 5.3 months. The three cases of megakaryoblastic transformation represented a significant proportion of all CGL blastic transformation cases (ten cases) evaluated by bone marrow examination in our institution during a 13-month period. Megakaryoblastic transformation of CGL may occur more frequently than has been appreciated, and can present as lytic bone lesions or as soft tissue megakaryoblastic sarcomas.
报告了3例慢性粒细胞白血病（CGL）巨核母细胞转化病例。在每例中，白血病转化均具有提示巨核细胞分化的形态学特征。这通过用针对血小板和巨核细胞糖蛋白IIb/IIIa抗原的单克隆抗体（HP1-1D）进行阳性免疫染色得以证实，在所有3例中大多数白血病原始细胞均表达该抗原。排除了白血病转化有多种系分化证据的病例。两名患者的一个显著特征是在就诊时出现溶骨性骨病变和软组织肿块。对一名患者的溶骨性骨病变和软组织肿块进行活检，发现有巨核母细胞白血病浸润，通过免疫细胞化学染色，其对巨核细胞标志物糖蛋白IIb/IIIa抗原和因子VIII（血管性血友病因子）抗原呈阳性。与粒细胞肉瘤不同，巨核母细胞肉瘤在细胞化学上对氯乙酸酯酶不着色。急性转化后的平均生存期为5.3个月。在我们机构13个月期间通过骨髓检查评估的所有CGL原始细胞转化病例（10例）中，这3例巨核母细胞转化占了相当大的比例。CGL的巨核母细胞转化可能比人们认识到的更为常见，并且可表现为溶骨性骨病变或软组织巨核母细胞肉瘤。
