Khaspekova S G, Golovkina L L, Donush E K, Golubeva N V, Shustova O N, Mazurov A V
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):65-69. doi: 10.26442/terarkh201890765-69.
Mechanisms underlying the development of neonatal alloimmune thrombocytopenia (NAIT) in in Russia have been studied.
Genetic polymorphisms of human platelet alloantigens (HPA) -1, -2, -3, -4, -5, and -15 were evaluated in 27 families having the newborns with NAIT. NAIT was diagnosed according to the following criteria: (1) newborn with thrombocytopenia; (2) mother with no thrombocytopenia and no increase of platelet associated IgG, (3) presence of antibodies reacting with paternal platelets in maternal plasma / serum. HPA genotyping revealed incompatibilities in 23 out of 27 tested families. In these 23 families HPA-1 conflicts were detected in 16 ones (70%). In 8 cases mothers were homozygous carriers of rare HPA-1b allele and in another 8 cases - of HPA-1a allele which cased incompatibilities with fetal HPA-1a and HPA-1b respectively. In 5 out of 23 families (22%) there were incompatibilities with fetal HPA-15 (HPA-15a, n=2 and HPA-15b, n=3), in 1 family - with HPA-5b (4%), and in 1 family - with HPA-3b (4%) alloantigens.
In conclusion the main causes of NAIT in Russia were HPA-1a and -1b conflicts and HPA-15 conflicts were the second frequent ones.
对俄罗斯新生儿同种免疫性血小板减少症（NAIT）发病机制进行了研究。
对27个有患NAIT新生儿的家庭进行了人类血小板同种抗原（HPA）-1、-2、-3、-4、-5和-15的基因多态性评估。NAIT根据以下标准诊断：（1）新生儿血小板减少；（2）母亲无血小板减少且血小板相关IgG未升高；（3）母体血浆/血清中存在与父亲血小板反应的抗体。HPA基因分型显示，在27个检测家庭中有23个存在不相容性。在这23个家庭中，16个（70%）检测到HPA-1冲突。8例母亲是罕见HPA-1b等位基因的纯合携带者，另外8例是HPA-1a等位基因的纯合携带者，分别与胎儿的HPA-1a和HPA-1b不相容。在23个家庭中的5个（22%），存在与胎儿HPA-15（HPA-15a，n = 2；HPA-15b，n = 3）的不相容性，1个家庭（4%）与HPA-5b不相容，1个家庭（4%）与HPA-3b同种抗原不相容。
总之，俄罗斯NAIT的主要原因是HPA-1a和-1b冲突，HPA-15冲突是第二常见原因。
