巨核细胞和血小板在骨髓增生性疾病中的作用

MD Jean Briere (Professor of Medicine, Head), MD Jean-Jacques Kiladjian (Assistant), MD Edith Peynaud-Debayle (Assistant)
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引用次数: 11

摘要

骨髓中巨核细胞(MK)增殖增加是三种ph阴性骨髓增殖性疾病(MPDs)的共同特征,即原发性血小板增多症(ET)、真性红细胞增多症(PV)和骨髓纤维化伴脾髓细胞化生(MMM),以及慢性髓细胞性白血病(CML)。具有多分叶细胞核和细胞聚集的mk增大是所有Ph阴性mpd的标志。基于X染色体失活的造血细胞克隆,现在可以在大多数女性患者的所有有核细胞部分以及血小板中进行研究。细胞荧光学研究表明PV和ET MK向更高倍性类别转变,这可能有助于区分原发性和反应性血小板增多症和CML患者,后者显着向较低的MK倍性值转变。MK增殖在MPDs常见的骨髓纤维化进化中的作用已经被明确确立。血小板衍生生长因子(PDGF)在骨髓纤维化中的意义已经得到证实。最近,MK合成和分泌的转化生长因子β (TGFβ)与成纤维细胞刺激有关。在mpd中反复观察到循环MK集落形成单位(CFU-MK)的显著增加,并且在大多数ET患者中自发形成MK集落。血小板生成素(TPO)的超敏性与TPO- mpl通路的功能缺陷有关,可能在自发性MK生长中起主要作用。目前还没有可用的血小板功能测试能够预测MPD患者发生血栓性或出血性并发症的风险。然而,血小板激活在缺血性红斑性肢痛症发病机制中的作用已经确立,并且发现出现出血表现与血小板计数超过1000 × 109/l之间存在相关性。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
4 Megakaryocytes and platelets in myeloproliferative disorders

Increased megakaryocyte (MK) proliferation in bone marrow is a feature common to the three Ph-negative myeloproliferative disorders (MPDs), i.e. essential thrombocythaemia (ET), polycythaemia vera (PV), and myelofibrosis with splenic myeloid metaplasia (MMM), and to chronic myelocytic leukaemia (CML). Enlarged MKs with multilobulated nuclei and cell clustering in close proximity are the hallmark of all the Ph negative MPDs. Clonality of haematopoietic cells, based on X chromosome inactivation, can now be studied in a majority of female patients in all nucleated cell fractions as well as in platelets. Cytofluorometric studies have demonstrated a shift towards higher ploidy classes in PV and ET MKs which may be useful in discriminating between both primary and reactive thrombocytosis and CML patients which show a significant shift to lower MK ploidy values.

The role of MK proliferation on the evolution of myelofibrosis common to MPDs has been firmly established. Implication of platelet-derived growth factor (PDGF)in myelofibrosis has already been demonstrated. More recently transforming growth factor β (TGFβ) synthesized and secreted by MK has been implicated in fibroblasts stimulation.

A significant increase in circulating colony-forming units of MKs (CFU-MK) has been repeatedly observed in MPDs as well as a spontaneous MK colony formation in a majority of ET patients. Hypersensitivity to thrombopoietin (TPO) in relation to a functional defect of the TPO-MPL pathway may play a major role in spontaneous MK growth.

There is no currently available test of platelet functions able to predict the risk of occurrence of thrombotic or haemorrhagic complications in MPD patients. However, the role of platelet activation in the pathogenesis of ischaemic erythromelalgia has been established and a correlation between presenting haemorrhagic manifestations and platelet counts in excess of 1000 × 109/l has been found.

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