Pregnancy Associated Thrombotic Microangiopathy
Journal Title: People's Journal of Scientific Research. - Year 2016, Vol 9, Issue 2
Abstract
he thrombotic microangiopathies (TMA) are a group of common microvascular occlusive disorders characterized by thrombocytopenia, microangiopathic hemolysis and multiorgan dysfunction. The pathological features are vascular damage manifested by arteriolar and capillary thrombosis with characteristic abnormalities in the endothelium and vessel wall. Pregnancy associated TMA is a life threatening rare disease reported to occur in approximately 1 in 25000 pregnancies, can occur at any stage of pregnancy and can be a key feature of several pregnancy related disorders such as thrombotic thrombocytopenic purpura (TTP ) / Haemolytic uremic syndrome (HUS), congenital TTP(CTTP), HELLP syndrome, or acute fatty liver (AFL). The pathogenesis of inherited (Upshaw–Schulman syndrome) and idiopathic TTP is related to a deficiency of, or antibodies to, a metalloprotease that cleaves vWF and ADAMTS13, respectively. The role of complement factor H (CFH) dysregulation has been reported in cases of pregnancy related HUS. Therapy is specific for each disease entity ranging form plasma exchange (PEX) in TTP /HUS and termination of pregnancy or delivery in HELLP syndrome. The likelihood of survival in patient cases is as high as 80–90% with early diagnosis and aggressive treatment using PEX. The chance of missing the cases is high because of the rarity of these disorders and diagnostic dilemmas. The patients need comprehensive care in close conjunction with physicians and obstetricians. The purpose of this review is to provide a prospective of pregnancy associated TMA and management options available to reduce maternal morbidity and mortality
Authors and Affiliations
Manisha Shrivastava, , Nehal Shah
Keywords
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