Radaelli Franca, Mazza Rita, Curioni Elisabetta, Ciani Alberto, Pomati Mauro, Maiolo Anna-Teresa
Department of Haematology, Institute of Medical Science, University of Milan, Ospedale Maggiore-IRCCS, Via F. Sforza 35, 20122 Milan, Italy.
Eur J Haematol. 2002 Aug;69(2):108-11. doi: 10.1034/j.1600-0609.2002.02734.x.
Essential thrombocythemia (ET) is a chronic myeloproliferative disorder that can rarely undergo leukemic transformation either in treated (3-7%) or untreated patients (1%). Evolution to myeloblastic or myelomonoblastic acute leukemia is commonly described in the literature, whereas lymphatic and megakaryocytic forms are considered unusual. Here, we report three cases of acute megakaryocytic leukemia (LMA-M7) among 11 acute leukemic transformations observed in our series of 321 ET patients. LMA-M7 was diagnosed employing immunophenotyping according to FAB criteria. These recurrences of LMA M7 suggest that this kind of evolution cannot be considered rare or casual in ET.
原发性血小板增多症(ET)是一种慢性骨髓增殖性疾病,在接受治疗的患者(3%-7%)或未接受治疗的患者(1%)中很少发生白血病转化。文献中通常描述其向成髓细胞性或粒单核细胞性急性白血病的演变,而淋巴样和巨核细胞形式则被认为不常见。在此,我们报告在我们的321例ET患者系列中观察到的11例急性白血病转化中有3例急性巨核细胞白血病(LMA-M7)。LMA-M7根据FAB标准采用免疫表型分析进行诊断。这些LMA-M7的复发表明,这种演变在ET中不能被视为罕见或偶然。