Butina E V, Zaitseva G A
Klin Lab Diagn. 2016 Oct;61(10):715-9.
The thrombocytopenia is found in 1%-5% of newborns. Depending on mechanisms of pathogenesis of thrombocytopenia is divided on immune and non-immune one. The reaction of of interaction between antibodies and antigens of superficial structures of cells are in the basis of immune destruction of thrombocytes. During intrauterine period and period of newborness auto-, trans- and alloimmune alternatives of development of thrombocytopenia can be observed. The neonatal alloimmune thrombocytopenia is registered with rate of 1 case per 800-1000 newborns. The study was targeted to developing algorithm of diagnostic of immune thrombocytopenia, detecting main diagnostic criteria, exploring clinical significance of results of laboratory tests. The methods of study included typing of genes of HPA system using polymerase chain reaction with detection of results in real-time mode, detection of compatibility of HPA-genotypes of mother and child using flow cytometry technique. The following criteria of diagnostic of neonatal alloimmune thrombocytopenia: 1. detection of incompatible combination of HPA genes in mother and child (HPA-1bb/HPA-1ab; HPA-5aa/HPA-5ab; HPA-15ab/HPA- 15ab); 2. detection in blood serum of mother antibodies adsorbing more than on 3% of thrombocytes of child; 3. absence of antithrombocyte antibodies in mother and child (coefficient of auto-sensitization is less than 5%). The immune genesis of thrombocytopenia is established in 40% 0f children with low number of thrombocytes at birth. In 50% of cases the cause was determined as anti-thrombocyte alloantibodies with diagnosis neonatal alloimmune thrombocytopenia. Also in 50% of cases decreasing of number of thrombocytes in children occurred as result of impact of autoantibodies of mother with diagnosis “transimmune thrombocytopenia”. The considered laboratory methods have high specificity and permit to properly diagnose immune causes of thrombocytopenia in newborns.
1%-5%的新生儿会出现血小板减少症。根据血小板减少症的发病机制，可分为免疫性和非免疫性。抗体与细胞表面结构抗原之间的相互作用反应是血小板免疫破坏的基础。在子宫内和新生儿期，可观察到血小板减少症发展的自身免疫、同种免疫和异种免疫替代情况。新生儿同种免疫性血小板减少症的发病率为每800-1000例新生儿中有1例。该研究旨在制定免疫性血小板减少症的诊断算法，确定主要诊断标准，探讨实验室检查结果的临床意义。研究方法包括使用聚合酶链反应并实时检测结果来对HPA系统基因进行分型，使用流式细胞术技术检测母婴HPA基因型的相容性。新生儿同种免疫性血小板减少症的诊断标准如下：1. 检测到母婴HPA基因不相容组合（HPA-1bb/HPA-1ab；HPA-5aa/HPA-5ab；HPA-15ab/HPA-15ab）；2. 在母亲血清中检测到吸附超过3%儿童血小板的抗体；3. 母婴体内无抗血小板抗体（自身致敏系数小于5%）。出生时血小板数量低的儿童中，40%的血小板减少症病因确定为免疫性。在50%的病例中，病因确定为抗血小板同种抗体，诊断为新生儿同种免疫性血小板减少症。另外，在50%的病例中，儿童血小板数量减少是由于母亲自身抗体的影响，诊断为“同种免疫性血小板减少症”。所考虑的实验室方法具有高特异性，能够正确诊断新生儿血小板减少症的免疫病因。
