Unfolding the pathophysiology of congenital thrombotic thrombocytopenic purpura in pregnancy: Lessons from a cluster of familial cases

Thrombotic Thrombocytopenic Purpura (TTP), a rare, potentially life-threatening thrombotic microangiopathy, manifests as both congenital (cTTP) and acquired forms. It is caused by the absence of or severe depletion in a disintegrin and metalloproteinase with thrombospondin motifs 13 (ADAMTS13) protease leading to the accumulation of ultra large von Willebrand Factor (vWF) multimers and extensive platelet adhesion and clumping, which can ultimately result in severe secondary end-organ damage. Pregnancy can provoke or exacerbate TTP leading to maternal and fetal complications.

Source: American Journal of Obstetrics and Gynecology - February 20, 2021 Category: OBGYN Authors: Shayna MIODOWNIK, Oleg PIKOVSKY, Offer EREZ, Yarden KEZERLE, Oleg LAVON, Anat RABINOVICH Tags: Original Research: Obstetrics Source Type: research