Shibata K, Shimamoto Y, Suga K, Sano M, Matsuzaki M, Yamaguchi M
Department of Internal Medicine, Saga Medical School, Japan.
Acta Haematol. 1994;91(2):84-8. doi: 10.1159/000204260.
Essential thrombocythemia (ET), one of the chronic myeloproliferative disorders, is a clonal disorder of multipotent stem cells. Although most patients with ET have a prolonged benign course, a minority of patients may develop a blastic crisis similar to chronic myelogenous leukemia (CML). A case of ET terminating in blastic crisis 8 years after the initial diagnosis is presented. The blast cells were cytochemically and immunophenotypically consistent with the acute myelogenous leukemia with minimal myeloid differentiation subtype of the FAB classification. From the review of the literature on blastic transformation of ET, acute leukemia with an M4 or M7 phenotype occurred more frequently. In addition, three valuable factors to predict the leukemic transformation of ET appear to be karyotypic abnormalities, such as involvement of chromosome 21, previous therapies with a mutagenic potential, and the capability of bone marrow cells to form in vitro spontaneous colonies as in CML.
原发性血小板增多症（ET）是慢性骨髓增殖性疾病之一，是一种多能干细胞的克隆性疾病。虽然大多数ET患者病程呈良性且持续时间长，但少数患者可能会发生类似于慢性粒细胞白血病（CML）的急变期。本文报告1例ET患者，初诊8年后发生急变期。原始细胞的细胞化学和免疫表型与FAB分类中最小髓系分化亚型的急性髓系白血病一致。从关于ET急变的文献回顾来看，具有M4或M7表型的急性白血病更为常见。此外，预测ET白血病转化的三个重要因素似乎是核型异常，如21号染色体受累、既往具有潜在诱变作用的治疗，以及骨髓细胞像CML那样在体外形成自发集落的能力。
