Thrombotic microangiopathies during pregnancy: The obstetrical and neonatal perspective

Abstract

Thrombotic Microangiopathies during pregnancy and puerperium are very rare and, if undiagnosed, can be lifethreating. Pregnancy and postpartum can represent a trigger in predisposed patients. Therefore, obstetricians are usually the first to observe clinical symptoms and laboratory abnormalities suggestive of Thrombotic Microangiopathies. The aim of this review is to briefly describe the obstetrical and perinatal outcome of these entities and highlight the clues for a correct diagnosis of pregnancy-related Thrombotic Microangiopathies.

Acute kidney injuries induced by thrombotic microangiopathy following severe hemorrhage in puerperants: A case series and literature review

“…TTP is a rare, life-threatening TMA characterized by a severe de ciency in ADAMTS-13 (A Disintegrin And Metalloprotease with ThromboSpondin type 1 domain 13) [29]. Pregnancy is a known trigger of TTP [1][2][3][30][31][32]. However, TTP was excluded by normal ADAMTS13 in these three patients [33], and complement-mediated thrombotic microangiopathy (C-TMA) also known as atypical hemolytic-uremic syndrome was considered.…”

“…The clinical consequences of TMA are thrombocytopenia, mechanical hemolytic anemia, and ischemic injuries to different organs, especially in the kidneys. TMAs are rare, severe conditions associated with serious morbidity and up to 90% mortality rate, if left untreated [3]. Pregnancy is a high-risk period for women to develop various types of TMA.…”

Thrombotic microangiopathy in pregnancy: when you hear hoofbeats, consider the zebras?

“…Thrombotic microangiopathy (TMA) refers to a set of conditions marked by an underlying pathologic alteration of the microvasculature, characterised by endothelial engorgement and platelet aggregation resulting in damage to the microcirculation 1 . Clinically, TMA manifests through microangiopathic hemolytic anaemia (MAHA) with evidence of red blood cell fragmentation, thrombocytopenia and micro‐thrombotic end‐organ ischemic injury 1,2 .…”

“…Thrombotic microangiopathy (TMA) refers to a set of conditions marked by an underlying pathologic alteration of the microvasculature, characterised by endothelial engorgement and platelet aggregation resulting in damage to the microcirculation 1 . Clinically, TMA manifests through microangiopathic hemolytic anaemia (MAHA) with evidence of red blood cell fragmentation, thrombocytopenia and micro‐thrombotic end‐organ ischemic injury 1,2 . The presence of TMA in the context of pregnancy should prompt consideration of three entities: (i) Pre‐eclampsia/HELLP (hemolysis, elevated liver enzymes and low platelets) syndrome; (ii) thrombotic thrombocytopenia purpura (TTP); and (iii) pregnancy‐associated aHUS (p‐aHUS), a complement‐mediated microangiopathy 3 …”

“…Pre‐eclampsia/HELLP is the most common TMA encountered in pregnancy, complicating 2–7% and 0·2–0·6% of gestations, respectively 1,4 . It is thought to be driven by abnormal placentation, endothelial dysfunction and imbalance of antiangiogenic factors present as early as the first trimester, yet its phenotypic variability remains poorly understood 1 . Moreover, recent studies have linked upregulation of the complement alternative pathway to the pathogenesis of HELLP 5,6 .…”