Cortelazzo S, Viero P, Bassan R, Barbui T
Ric Clin Lab. 1981 Jan-Mar;11(1):35-42. doi: 10.1007/BF02886685.
Several platelet abnormalities have been described in myeloproliferative diseases. The present study deals with 81 patients with polycythaemia vera, chronic myelogenous leukemia, essential thrombocythaemia and idiopathic myelofibrosis, and reports the analysis of the findings in platelet-induced aggregation. Platelet abnormalities induced by ADP, adrenaline and collagen were found in 41.9% of the patients. A defect of primary aggregation was documented in 13 cases and one of them showed an aggregation pattern similar to that of Glanzmann's disease. Fifteen patients had an impairment of secondary aggregation, and in one case of this group the platelet malondialdehyde and serotonin findings were consistent with a defect resembling that of typical congenital storage pool deficiency. A disturbance of the arachidonic acid pathways associated with a storage pool deficiency was found in a third patient belonging to a group with abnormalities of primary and secondary aggregation. In conclusion, platelets in myeloproliferative diseases have several defects and in a few cases their combination is similar to those of congenital diseases.
在骨髓增殖性疾病中已描述了几种血小板异常情况。本研究涉及81例真性红细胞增多症、慢性粒细胞白血病、原发性血小板增多症和特发性骨髓纤维化患者，并报告了对血小板诱导聚集结果的分析。41.9%的患者发现了由二磷酸腺苷（ADP）、肾上腺素和胶原诱导的血小板异常。记录到13例原发性聚集缺陷，其中1例显示出与Glanzmann病相似的聚集模式。15例患者继发性聚集受损，该组中的1例患者血小板丙二醛和血清素的检测结果与典型先天性贮存池缺乏症的缺陷一致。在1例原发性和继发性聚集异常的患者中发现与贮存池缺乏相关的花生四烯酸途径紊乱。总之，骨髓增殖性疾病中的血小板存在多种缺陷，少数情况下其组合与先天性疾病相似。
