Hernández J M, San Miguel J F, González M, Orfao A, Cañizo M C, Bascones C, Hernández J, López Borrasca A
Servicio de Hematología, Hospital Clínico, Salamanca, Spain.
J Clin Pathol. 1992 May;45(5):427-30. doi: 10.1136/jcp.45.5.427.
To determine the characteristics of blastic transformation of idiopathic myelofibrosis.
The clinical and haematological features, as well as the morphological characteristics of blast cells, were analysed in nine adults with blast transformation.
Most of the patients were male and had enlarged spleens and livers. Five of the patients had normal platelet counts, while all had pronounced anaemia and a moderate degree of leucocytosis. The duration of the acute phase was usually short: 16 (SD 8) weeks. Most myeloid cell lineages--granulocytic, monocytic, and megakaryocytic--were similarly distributed. One patient also had a hybrid (lymphoid-myeloid) phenotype. The morphological assessment of blast cells agreed with immunophenotyping in five out of the nine cases. The onset of the blastic phase was not related to previous treatment.
A pluripotential stem cell with preferential myeloid commitment would be the target cell of blast transformation in idiopathic myelofibrosis. Our immunophenotypic data do not support the concept of a preferential association between megakaryocytic lineage and the acute transformation of idiopathic myelofibrosis. The absence of previous treatment in some cases suggests that this kind of evolution is part of the natural history of idiopathic myelofibrosis.
确定原发性骨髓纤维化原始细胞转化的特征。
对9例发生原始细胞转化的成年患者的临床和血液学特征以及原始细胞的形态学特征进行分析。
大多数患者为男性，脾脏和肝脏肿大。5例患者血小板计数正常，而所有患者均有明显贫血和中度白细胞增多。急性期持续时间通常较短：16（标准差8）周。大多数髓系细胞谱系——粒细胞系、单核细胞系和巨核细胞系——分布相似。1例患者还具有混合（淋巴-髓系）表型。9例中有5例原始细胞的形态学评估与免疫表型分析结果一致。原始细胞期的发病与既往治疗无关。
具有优先髓系分化倾向的多能干细胞可能是原发性骨髓纤维化原始细胞转化的靶细胞。我们的免疫表型数据不支持巨核细胞系与原发性骨髓纤维化急性转化之间存在优先关联的概念。某些病例中既往未接受治疗提示这种演变是原发性骨髓纤维化自然病程的一部分。
