血小板增多的慢性骨髓增殖性疾病中的血小板α-颗粒释放

Platelet alpha-granule release in chronic myeloproliferative disorders with thrombocytosis.

作者信息

Sacchi S, Curci G, Piccinini L, Messerotti A, Cucci F, Bursi R, Zaniol P, Torelli U

出版信息

Scand J Clin Lab Invest. 1986 Apr;46(2):163-6. doi: 10.3109/00365518609083653.

Abstract

Platelet alpha-granule release in 22 patients affected by chronic myeloproliferative disorders with thrombocytosis and seven subjects with thrombocytosis secondary to splenectomy were studied. We found elevated beta-thromboglobulin (BTG) and platelet factor 4 (PF4) plasma levels in all patients. Intraplatelet content of BTG and PF4 was decreased in patients with idiopathic thrombocythaemia (IT) and idiopathic myelofibrosis (IMF). The BTG and PF4 results were also expressed as the ratio plasma BTG and PF4: whole blood platelet count. In patients with IT, BTG: whole blood platelet count ratio was low, conversely, the same ratio was high in patients with IMF. In conclusion, our results suggest the presence of an abnormal, BTG-deficient clone in IT and a peripheral platelet activation in IMF.

摘要

对22例患有慢性骨髓增殖性疾病并伴有血小板增多症的患者以及7例因脾切除继发血小板增多症的受试者的血小板α-颗粒释放情况进行了研究。我们发现所有患者的血浆β-血小板球蛋白（BTG）和血小板因子4（PF4）水平均升高。特发性血小板增多症（IT）和原发性骨髓纤维化（IMF）患者的血小板内BTG和PF4含量降低。BTG和PF4结果也表示为血浆BTG和PF4与全血血小板计数的比值。在IT患者中，BTG与全血血小板计数的比值较低，相反，在IMF患者中该比值较高。总之，我们的结果表明IT患者存在异常的、BTG缺乏的克隆，而IMF患者存在外周血小板活化。