Rabellino E M, Levene R B, Nachman R L, Leung L L
Blood. 1984 Mar;63(3):615-22.
Abnormal proliferation of the megakaryocytic line was observed in the marrow tissue from patients with myeloproliferative disorders. Megakaryocytes were identified by immunofluorescence using distinct platelet protein markers. Plasma factor VIII antigen (factor VIII:AGN) and platelet glycoproteins IIb and IIIa were detected in normal mature and early megakaryocytes, as well as in a morphologically heterogeneous population of low density marrow cells regarded as atypical megakaryocytes. Atypical megakaryocytes were defined as oval/round 14-35-micron diameter blast-like mononuclear/multinucleated cells bearing platelet protein markers with distinct morphological features, including cytoplasmic vacuolation, variable nuclear/cytoplasmic ratios, and variable cytoplasmic granulation. Atypical megakaryocytes were observed in most chronic myelogenous leukemia (CML) patients and in two patients with polycythemia vera, representing between 60 and 1,840 cells/10(4) cells (less than 1.050 g Percoll/cu cm). No atypical megakaryocytes were found in (a) 20 normal controls, (b) two patients with essential thrombocythemia, (c) a patient with thrombocytosis secondary to acute bleeding, and (d) in two patients with CML. Atypical megakaryocytes appear to represent a single-cell population, as demonstrated by a series of double immunofluorescence assays using combinations of five different antiplatelet protein sera. There was a statistically significant correlation between the frequency of atypical megakaryocytes and the presence of immature forms of myeloid cells in blood. Analyses of Fc IgG receptors conducted with two different immunofluorescence systems have demonstrated that phenotypic similarities existed between atypical megakaryocytes and myeloproliferative platelet proteins and differentiation markers on megakaryocytes are useful in elucidating the pathophysiologic alterations occurring in the megakaryocytic compartment in patients with myeloproliferative disorders.
在骨髓增殖性疾病患者的骨髓组织中观察到巨核细胞系的异常增殖。通过使用不同的血小板蛋白标志物进行免疫荧光鉴定巨核细胞。在正常成熟和早期巨核细胞中，以及在被视为非典型巨核细胞的形态学异质性低密度骨髓细胞群体中，检测到血浆因子VIII抗原（因子VIII:AGN）以及血小板糖蛋白IIb和IIIa。非典型巨核细胞被定义为直径14 - 35微米的椭圆形/圆形、类似原始细胞的单核/多核细胞，带有血小板蛋白标志物，具有独特的形态特征，包括细胞质空泡化、可变的核/质比以及可变的细胞质颗粒化。在大多数慢性粒细胞白血病（CML）患者和两名真性红细胞增多症患者中观察到非典型巨核细胞，其数量为每10⁴个细胞中有60至1840个细胞（Percoll密度小于1.050 g/立方厘米）。在以下情况中未发现非典型巨核细胞：（a）20名正常对照者；（b）两名原发性血小板增多症患者；（c）一名急性出血继发血小板增多症的患者；（d）两名CML患者。如使用五种不同抗血小板蛋白血清组合进行的一系列双重免疫荧光测定所示，非典型巨核细胞似乎代表一个单细胞群体。非典型巨核细胞的频率与血液中未成熟髓样细胞的存在之间存在统计学上的显著相关性。使用两种不同免疫荧光系统对Fc IgG受体进行的分析表明，非典型巨核细胞与骨髓增殖性血小板蛋白之间存在表型相似性，并且巨核细胞上的分化标志物有助于阐明骨髓增殖性疾病患者巨核细胞区室中发生的病理生理改变。
