Vlădăreanu Ana-Maria, Popov Viola, Bumbea H, Ciufu Cristina, Vasilache Veronica, Petre Anca, Onisâi Minodora
Facultatea de Medicină, Clinica de Hematologie SUUB, Universitatea de Medicini si Farmacie "C. Davila" Bucureşti.
Rev Med Chir Soc Med Nat Iasi. 2011 Jan-Mar;115(1):14-9.
Chronic myeloproliferative disorders (CMD) and Myelodisplastic Syndromes (MDS) represents a group of clonal pluripotent stem-cell pathologies. During their natural history, the clinical picture reveals both thrombosis and hemorrhage. The thrombosis could affect the microvessels, and also the large vessels, including even less usual territories (suprahepatic veins, porta vein, pulmonary vein). There are many factors contributing to thrombosis in myeloproliferative chronic disorders--the associated comorbidities, the numeric alterations of blood elements and also the disorders of the platelet's function. Thus, there were described quantitative and qualitative anomalies of platelet's receptors: GP Ib, GP IIb/IIIa, GP IV, GP VI, thrombopoietin receptor of the platelet cMPL, the increase of platelet activation; the increase of P selectin and thrombospondin and the increase on GP IIb/IIIa expression--they were all correlated with thrombosis. An important role has been attributed to JAK2 mutation, which affects the platelet receptor for thrombopoietin cMPL. Regarding the hemorrhage in chronic myeloproliferative syndrome, it is favored by many disorders in platelet's function, such as: the decrease of von Willebrand factor's receptor of the platelet, which leads to acquired Bernard Soulier syndrome; quantitative and qualitative disorders of dense granules of the platelet, decrease of the secretion and platelet aggregation after epinephrine, ADP and collagen stimulation. It was also described the acquired von Willebrand syndrome, most frequently type 2.
慢性骨髓增殖性疾病（CMD）和骨髓增生异常综合征（MDS）是一组克隆性多能干细胞疾病。在其自然病程中，临床表现既有血栓形成又有出血。血栓形成可累及微血管，也可累及大血管，甚至包括一些不太常见的部位（肝上静脉、门静脉、肺静脉）。骨髓增殖性慢性疾病中血栓形成有许多促成因素——相关合并症、血液成分的数量改变以及血小板功能障碍。因此，已描述了血小板受体的定量和定性异常：糖蛋白Ib（GP Ib）、糖蛋白IIb/IIIa（GP IIb/IIIa）、糖蛋白IV（GP IV）、糖蛋白VI（GP VI）、血小板cMPL的血小板生成素受体，血小板活化增加；P选择素和血小板反应蛋白增加以及GP IIb/IIIa表达增加——它们均与血栓形成相关。JAK2突变被认为起重要作用，其影响血小板生成素cMPL的血小板受体。关于慢性骨髓增殖综合征中的出血，血小板功能的许多紊乱会促发出血，例如：血小板的血管性血友病因子受体减少，导致获得性伯纳德·索利尔综合征；血小板致密颗粒的定量和定性紊乱，肾上腺素、二磷酸腺苷（ADP）和胶原刺激后分泌减少及血小板聚集减少。还描述了获得性血管性血友病综合征，最常见的是2型。
