Mazurov A V, Khaspekova S G, Vasiliev S A
National Medical Research Center for Cardiology Russian Ministry of Health, Moscow, Russia.
National Medical Research Center for Hematology Russian Ministry of Health, Moscow, Russia.
Ter Arkh. 2018 Aug 17;90(7):4-13. doi: 10.26442/terarkh20189074-13.
Laboratory methods used for the diagnostics of thrombocytopenias are reviewed. Differential diagnosis is usually carried out between immune and hypoproductive forms of thrombocytopenia. Immune thrombocytopenias are caused by appearance in blood of antiplatelet abtibodies and accelerated destruction of platelets sensibilized by those antibodies, and hypoproductive thrombocytopenias - by impaired platelet production in the bone marrow. Main directions of the laboratory diagnostics of thrombocytopenias - analysis of auto- and alloautoantibodies and evaluation of platelet production and turnover in the blood stream. The following methods are used for the investigation of antiplatelet antibodies: 1) measurement of platelet associated immunoglobulins; 2) determination of circulating antibodies reacting with platelets; 3) determination of antibodies using antigen specific methods - by their reactivity with isolated platelet antigens (glycoproteins). Efficacy of platelet production could be assessed by measuring in blood the amount of "young" (reticulated) platelets. One more method for the evaluation of platelet production as well as the rate of platelet turnover - measurement of plasma soluble glycocalicin, glycoprotein Ib fragment shed from the surface of platelets upon their destruction in spleen and liver. In patients with immune thrombocytopenia autoantibodies are evaluated in all cases, the percentage of reticulated platelets is significantly increased and the amount of plasma glycocalicin is within the normal range or increased. In patients with hypoproductive thrombocytopenia autoantibodies are not detected or detected at low level, the percentage of reticulated platelets is within the normal range or slightly increased and the amount of plasma glycocalicin is lowered. Diagnostics of hapten forms of immune thromocytopenias (heparin-induced thrombocytopenia and others) and of alloimmune thrombocytopenias (neonatal alloimmune thrombocytopenia in particular) are considered in the separate sections of this review.
本文综述了用于诊断血小板减少症的实验室方法。血小板减少症的鉴别诊断通常在免疫性和低生成性血小板减少症之间进行。免疫性血小板减少症是由血液中抗血小板抗体的出现以及这些抗体致敏的血小板加速破坏引起的，而低生成性血小板减少症则是由骨髓中血小板生成受损所致。血小板减少症实验室诊断的主要方向——分析自身和同种自身抗体以及评估血流中血小板的生成和周转率。以下方法用于检测抗血小板抗体：1）测量血小板相关免疫球蛋白；2）测定与血小板反应的循环抗体；3）使用抗原特异性方法测定抗体——根据其与分离的血小板抗原（糖蛋白）的反应性。血小板生成的功效可通过测量血液中“年轻”（网织）血小板的数量来评估。另一种评估血小板生成以及血小板周转率的方法——测量血浆可溶性糖萼蛋白，即血小板在脾脏和肝脏中破坏时从其表面脱落的糖蛋白Ib片段。在免疫性血小板减少症患者中，所有病例均评估自身抗体，网织血小板百分比显著增加，血浆糖萼蛋白量在正常范围内或增加。在低生成性血小板减少症患者中，未检测到自身抗体或检测到的水平较低，网织血小板百分比在正常范围内或略有增加，血浆糖萼蛋白量降低。本综述的单独章节中考虑了免疫性血小板减少症的半抗原形式（肝素诱导的血小板减少症等）和同种免疫性血小板减少症（特别是新生儿同种免疫性血小板减少症）的诊断。
