الفهرس | Only 14 pages are availabe for public view |
Abstract Thrombotic microangiopathies (TMA) can be a feature of several pregnancies related disorders such as Haemolytic uremic syndrome (HUS), thrombotic thrombocytopenic purpura (TTP), HELLP syndrome, acute fatty liver (AFL) and catastrophic antiphospholipid syndrome (CAPS). It encompasses a spectrum of different disorders with a similar pathogenesis, but in most of the cases completely different therapy. It can take several days to obtain the diagnosis, and in case of doubt therapeutic plasma exchange (TPE) should be started immediately to ensure better outcome. By measuring (ADAMTS13) activity, it may be possible to distinguish between the different causes of TMA. Pregnancy-related TMA can occur before or after birth. A Pregnancy-related TMA that develops during the puerperium, typically develops about the fourth day postpartum. No other significant differences are seen between antepartum and postpartum pregnancy related TMA. In critically ill patients it may be difficult to distinguish TMA from disseminated intravascular coagulation (DIC). DIC is associated with prolongation of global clotting times, prothrombin time and activated partial thromboplastin time due to consumption of clotting factors. TMA occurs by activation of platelets (congenital or acquired abnormalities of ADAMTS13), and by primary endothelial injury as with HELLP syndrome. Antepartum pregnancy-related TMA usually occurs at 28 ± 8 weeks of pregnancy. Therapeutic approaches in TMA associated with pregnancy are different and survival of patients depends on a timely diagnosis. Clinical vigilance requires for early detection of TMA. After that is necessary as soon as possible investigate the ADAMTS13 level to avoid TTP. Plasma exchange may be first urgent step of treatment in all cases of TMA, but after clarifying the diagnosis therapy should be adjusted. Timely diagnosis and effective treatment including early prescribed targeted therapy in aHUS patients can improve the survival rate and outcomes. Early recognition and aggressive therapy with PEX, plasma infusion and termination of pregnancy or successful delivery, (depending upon pregnancy associated TMA), can reduce mortality and morbidity in pregnant women to a great extent. |