Zuk E
Kliniki Hematologii, Pomorskiej Akademii Medycznej w Szczecinie.
Ann Acad Med Stetin. 1998;44:137-54.
Symptoms of hemorrhagic diathesis and/or thrombotic complications are observed in patients with myeloproliferative syndrome (m-p.s.). It is emphasized that there is a meaningful direct and indirect share of blood platelets in the mentioned disturbances of hemostasis. Therefore in own investigations an attempt was made to evaluate the morphology and function of blood platelets in patients with various forms of m-p.s., as well as to find an answer to the question: do any differences in morphology and function (activation) of blood platelets appear in patients with m-p.s.?, and is activation of blood platelets always accompanied by the symptom of hemorrhagic diathesis or past thrombotic episodes (the second ones evidenced in the patients anamneses)?, do the blood platelets in idiopathic thrombocytopenia (one of the forms of m-p.s.), differ morphologically and functionally from platelets in other forms of m-p.s. The investigation comprised 45 patients, in whom m-p.s. was diagnosed at Department of Hematology PAM (28 women, 17 men) aged from 36 to 82 years (mean age 58.10 + 10.61). The diagnosis of m-p.s. in each patient was established on the basis of clinical picture and laboratory examinations (blood morphology, hematocrit, FAG score, histopathological examination of bone marrow). In patients the accomplished determination concerned blood platelets count, mean blood platelet volume, platelocrit, volume range of blood platelets, coagulation time by Duke's method. The function of platelets was estimated with the help of the following studies: aggregation of blood platelets under the influence of ADP according to Born's method, concentration of beta-thromboglobulin (beta-TG) and platelet factor--4 (PF-4) in plasma by ELISA method. In order to pay due consideration to the effect of blood platelets count exerted on the concentration of beta-TG and PF-4, a supplementary parameter was introduced, the so-called beta-TG and PF-4 standardized. That allowed for studying the platelets in all patients under the same conditions. The results of own investigations have shown that most of the patients with m-p.s. had disturbances involving the morphology of blood platelets (Tab. 2) and increased activity of platelets, being expressed by a rise in concentrations of beta-TG and PF-4 in plasma (Fig. 1, 2) and by abnormal aggregation of platelets due to ADP influence. Moreover, the determination of beta-TG and PF-4 concentration in plasma in thrombocytopenia states, particularly in patients with idiopathic thrombocytopenia ought to be performed after the standardization of platelets count, the finding being also supported by the results of own investigations (Tab. 3). There may be compliance with the viewpoint expressed in the literature that the results of studying the morphological parameters of blood platelets and their function (activation) in m-p.s. are not a prognostic factor for the appearance of thrombotic complications and/or hemorrhages.
骨髓增殖综合征(m-p.s.)患者会出现出血素质和/或血栓形成并发症的症状。需要强调的是,血小板在上述止血障碍中有着重要的直接和间接作用。因此,在我们自己的研究中,试图评估各种形式m-p.s.患者血小板的形态和功能,并回答以下问题:m-p.s.患者的血小板形态和功能(激活)是否存在差异?血小板激活是否总是伴有出血素质症状或既往血栓形成发作(后者可在患者既往史中得到证实)?特发性血小板减少症(m-p.s.的一种形式)患者的血小板在形态和功能上是否与其他形式m-p.s.患者的血小板不同?该研究包括45例患者,他们在波兰军队医学科学院血液科被诊断为m-p.s.(28名女性,17名男性),年龄在36至82岁之间(平均年龄58.10±10.61)。每位患者的m-p.s.诊断是基于临床表现和实验室检查(血液形态学、血细胞比容、FAG评分、骨髓组织病理学检查)确定的。对患者进行的测定包括血小板计数、平均血小板体积、血小板压积、血小板体积范围、杜克法凝血时间。借助以下研究评估血小板功能:根据博恩方法测定ADP作用下血小板的聚集情况,采用ELISA法测定血浆中β-血小板球蛋白(β-TG)和血小板因子4(PF-4)的浓度。为了充分考虑血小板计数对β-TG和PF-4浓度的影响,引入了一个补充参数,即所谓的标准化β-TG和PF-4。这使得能够在相同条件下研究所有患者的血小板。我们自己的研究结果表明,大多数m-p.s.患者存在涉及血小板形态的紊乱(表2)以及血小板活性增加,表现为血浆中β-TG和PF-4浓度升高(图1、2)以及ADP影响导致的血小板异常聚集。此外,在血小板减少状态下,特别是在特发性血小板减少症患者中,血浆中β-TG和PF-4浓度的测定应在血小板计数标准化后进行,我们自己的研究结果(表3)也支持这一发现。文献中表达的观点可能是正确的,即研究m-p.s.患者血小板形态参数及其功能(激活)的结果并非血栓形成并发症和/或出血出现的预后因素。