Гемостаз-одна из защитных систем организма, обеспечивающая, с одной стороны, сохранение крови в кровеносном русле в жидком агрегатном состоянии, а с другой-образование тромбов при повреждении стенки сосудов для остановки кровотечения и предотвращения кровопотери. В то же время это одна из «проблемных» систем человеческого организма. Термины «гемостаз» и «свертывание» часто используют как синонимы, однако это не вполне корректно [1]. Гемостаз-комплексный и сложный процесс, требующий взаимодействия многочисленных физиологических механизмов. Он разделяется на первичный (эндотелиальные клетки и тромбоциты) и вторичный (плазменные факторы). Соответствующие клеточные и молекулярные механизмы направлены на «опечатывание» поврежденных сосудов с локальным образованием сгустков, предотвращающих кровотечение [2]. Как только целостность сосуда восстанавливается, происходит разрушение тромба и возобновляется нормальный гемостаз. Дисбаланс между составляющими гемостаза может произойти в периоперационном периоде или во время критической болезни, приводя к увеличенному риску тромбоза, кро-вотечения или, в некоторых случаях, обоих осложнений. Поэтому понимание нормальных физиологических процессов важно для определения терапевтической тактики модуляции кровотечения и тромбоза. Нарушение сбалансированности между ее составляющими даже при различных физиологических состояниях (беременность, кровопотеря и т. д.), а тем более при различных патологических процессах (травмы, инфекции, онкологические, аутоиммунные заболевания) является причиной либо кровотечения, либо тромбоза. Физиология плазменного гемостаза Условно систему свертывания крови можно подразделить на три компонента: свертывание, противосвертывание и фибринолиз [3]. Условно, так как в действительности эти системы тесно взаимосвязаны, а их разделение является лишь способом изложения материала. Существует несколько теорий свертывания крови: классическая теория свертывания; теория водопада, или каскадная теория; пересмотренная теория коагуляции; клеточная модель гемостаза [1, 2].

Вопросы по критериальным признакам антифосфолипидного синдрома (АФС), в частности о необходимости включения некоторых клинических проявлений, а также серологических маркёров, отличных от классических, остаются актуальными. В обзоре приведены предлагаемые профили некритериальных проявлений АФС, обсуждается необходимость пересмотра классификационных критериев АФС. Today questions about the antiphospholipid syndrome (APS) criteria remain actual. This applies in particular about the need to include some clinical manifestations, as well as serological markers that are distinguishable from those classic ones, in APS criteria. The review presents the proposed profiles of non-criterial APS manifestations, and discusses the need to revise APS classification criteria.

Objective: To define the role of IgA antibodies to cardiolipin (aCL) and IgA antibodies to beta-2 glycoprotein 1 (anti-β2-GP1) in the development of vascular complications in patients with antiphospholipid syndrome (APS) and systemic lupus erythematosus (SLE). Material and methods: A total of 187 patients with one of the following diagnoses: primary APS (PAPS), probable APS, SLE with APS, and SLE without APS. The comparison group consisted of 49 patients with other rheumatic diseases (RD), the control group included 100 relatively healthy individuals (without RD, oncological pathology, and infectious diseases). All patients underwent standard clinical, laboratory, and instrumental examinations before being included in the study and during follow-up. The aPL study included the determination of IgG/IgM aCL, IgG/IgM anti-β2-GP1 by enzyme-linked immunosorbent assay (ELISA), IgG/IgM/IgA aCL, IgG/IgM/IgA anti-β2-GP1 by chemiluminescence analysis (CLA), and lupus anticoagulant (LA). Results: IgA aCL were detected in 75 (40%) of the 187 patients with APS and SLE, in none of the comparison group, and in 2 (2%) of the control one. IgA anti-β2-GP1 were detected in 63 (34%) of the 187 patients with APS and SLE, in none of the patients in the comparison group, and in one (1%) of the control group. The prevalence of IgA aCL and IgA anti-β2-GP1 and their levels were statistically significantly higher in patients with APS (PAPS and SLE + APS) than the levels in patients with SLE and those of the comparison and control groups (p < 0.05). IgA aCL and IgA anti-β2-GP1 were significantly associated with thrombosis in APS (χ2 = 4.96; p = 0.02 and χ2 = 4.37; p = 0.04, respectively). The risk of thrombosis was 2.04 times higher in patients with positive IgA aCL than in patients without these antibodies, as well as in patients with positive IgA anti-β2-GP1; it was twice as high as in patients without antibodies. There was a high specificity of IgA aCL and IgA anti-β2-GP1 for both the diagnosis of APS and its clinical manifestations, despite a low sensitivity. Conclusions: The study revealed a relationship of thrombosis and APS with IgA aCL and IgA anti-β2-GP1. There was a high specificity of IgA aCL and IgA anti-β2-GP1 (95% and 93%, respectively) for the diagnosis of APS with a low sensitivity (54% and 44%, respectively). There were no patients with isolated positivity of IgA aCL and IgA anti-β2-GP1.

Aim. To determine the significance of antibodies to the phosphatidylserine/prothrombin complex (aPS/PT) in patients with systemic lupus erythematosus (SLE) antiphospholipid syndrome (APS). Materials and methods. A total of 190 patients were included in the study: 123 (64.7%) with reliable SLE and 55 (29%) with PAPS. The control group included 100 relatively healthy subjects of comparable age. All patients were tested for classical aPL as well as IgG/IgM-anti-PS/PT by enzyme immunoassay. Results. Based on the average values of IgG/IgM aPS/PT of the control group, the levels of positivity were allocated mean (M) + 3 or 5 standard deviations (SD): M+3SD and M+5SD. IgG aPS/PT levels above 73.6 U/ml (M+5SD) were more accurate diagnostic, for IgM aPS/PT above 18.0 U/ml. IgG-aPS/PT were detected in 84 (44%) of 190 patients. Levels above diagnostic levels were detected in 68 (65%) of 104 patients with APS (55 with PAPS and 59 with SLE+APS). Thrombosis was significantly more common in patients with IgG aPS/PT compared with patients negative for IgG aPS/PT. Arterial but not venous thrombosis was associated with IgG aPS/PT positivity. Conclusion. The frequency of detection of IgG aPS/PT in the examined patients was 44%, IgM aPS/PT 29% and their combination 19% of 190 patients. Half of the patients with probable APS had positive IgG aPS/PT and third IgM aPS/PT. Median IgG aPS/PT were significantly higher in patients with APS compared to patients without APS and the control group. Thrombosis was associated with IgG aPS/PT. Arterial thrombosis was significantly more frequently reported in patients with IgG aPS/PT. The sensitivity of IgG aPS/PT for reliable APS at levels greater than 73.6 units/ml was 59%, specificity 92%, for IgM aPS/PT 35% and 91%, respectively.