الفهرس 
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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.