Thiele J, Vykoupil K F, Georgii A
Virchows Arch A Pathol Anat Histol. 1980;389(3):287-305. doi: 10.1007/BF00430656.
The clinical and morphological findings are presented in two patients suffering from myelofibrosis and osteomyelosclerosis which terminated in an acute blastic crisis. Clinical follow-up data and light microscopy of the bone marrow however, revealed a chronic megakaryocytic-granulocytic myelosis (CMGM) with progression into myelofibrosis during the course of disease. In one patient the blastic transformation involved predominantly basophils, and in the other, neutrophils, with an accompanying abnormal proliferation of megakaryocytes in both cases. Electron microscopy of this cell population demonstrated remarkable atypicalities of the neutrophilic, basophilic and megakaryocytic cell lines. These abnormalities consisted of a nuclear-cytoplasmic asynchrony and a partial arrest of maturation, sometimes resulting in bizarre cell forms. Our investigations support the hypothesis of a mixed cellularity type of myelosis with a gradual and insiduous progression into osteomyelofibrosis/-sclerosis and a potential blastic crisis. In the evolution of blastic crisis all cell lines may be transformed, but with predominance of one population - basophils and neutrophils in our two cases - in addition to atypicalities of megakaryocytes.
两名患有骨髓纤维化和骨髓硬化症并最终发展为急性原始细胞危象的患者的临床和形态学表现如下。然而,临床随访数据和骨髓的光学显微镜检查显示为慢性巨核细胞-粒细胞性骨髓增生症(CMGM),在疾病过程中进展为骨髓纤维化。在一名患者中,原始细胞转化主要涉及嗜碱性粒细胞,而在另一名患者中则涉及中性粒细胞,且在两种情况下均伴有巨核细胞的异常增殖。对该细胞群体的电子显微镜检查显示嗜中性、嗜碱性和巨核细胞系存在明显的异常。这些异常包括核质不同步和成熟部分停滞,有时会导致怪异的细胞形态。我们的研究支持骨髓增生症的混合细胞类型假说,即逐渐隐匿地进展为骨髓纤维化/硬化症并可能发生原始细胞危象。在原始细胞危象的演变过程中,所有细胞系都可能发生转化,但在我们的两个病例中,除了巨核细胞异常外,以一个群体为主——嗜碱性粒细胞和中性粒细胞。