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作者报告了2例骨髓增生性疾病急变及最终成为巨核细胞白血病的动态临床表现,并对巨核细胞白血病与其他骨髓增生性疾病的关系及对巨核细胞白血病的治疗进行了讨论。例1、61岁,男,诊断为骨髓纤维化。由于血小板减少,血红蛋白减低,腹部不适,而行切脾。脾重2,235g,组织学特点为髓样化生。染色体研究,未见异常,亦朱见Ph~1染色体。白细胞硷性磷酸酶记分为243及198(正常15～70)。此后患者先以马利兰治疗,继因疾病发生急变改用阿糖胞苷及输血治疗。死前7个月血中开始出现小巨核细胞。临死前血小板计数超过10×10~(12)/L,并有很多不典型的巨型血小板。
The authors report two cases of myeloproliferative disorders and eventually the dynamic clinical manifestation of megakaryocyte leukemia and discuss the relationship between megakaryoblastic leukemia and other myeloproliferative diseases and the treatment of megakaryocytic leukemia. Example 1, 61 years old, male, diagnosed as myelofibrosis. Due to thrombocytopenia, hemoglobin decreased, abdominal discomfort, and cut the spleen. Spleen weight 2,235g, histological features of myeloid metaplasia. Chromosome studies, no abnormalities, also see Zhu Ph ~ 1 chromosome. Leukocyte alkaline phosphatase score 243 and 198 (normal 15 to 70). Since then, patients treated with Maryland first, followed by rapid change due to the disease with cytarabine and transfusion therapy. Seven months before the death of blood began to appear small megakaryocytes. Pre-mortem platelet count over 10 × 10 ~ (12) / L, and there are many atypical giant platelets.