Recommendations for the dia gnosis and treatment of pregnancy -⁠ and peripartum-associated thrombotic microangiopathies –⁠ a multidisciplinary consensus

Czech version

Authors: R. Ryšavá ¹; M. Koucký ²; O. Šimetka ³; P. Žák ⁴; J. Bláha ⁵; D. Frausová ¹; T. Indra ¹; J. Vojtěch ²; J. Gumulec ⁶
Authors‘ workplace: Klinika nefrologie 1. LF a VFN v Praze ¹; Klinika gynekologie, porodnictví a neonatologie 1. LF a VFN v Praze ²; Gynekologicko-porodnická klinika FN Ostrava ³; IV. interní hematologická klinika FN Hradec Králové ⁴; Klinika anesteziologie, resuscitace a intenzivní medicíny 1. LF a VFN v Praze ⁵; Klinika hematoonkologie LF OU FN Ostrava ⁶
Published in: Transfuze Hematol. dnes,31, 2025, No. Ahead of Print, p. 1-12.
Category: Best Practices
doi: https://doi.org/10.48095/cctahd2025prolekare.cz17

Overview

Thrombotic microangiopathy (TMA) is a very serious pathological condition, not only in obstetrics, which is associated with the formation of thrombosis at the capillary and arteriolar level as a result of endothelial damage and complement activation. It is accompanied by microangiopathic haemolytic anaemia (MAHA), thrombocytopenia, and dysfunction of various organs. In addition, it is relatively often associated with secondary systemic changes in coagulation. TMA encompasses a very heterogeneous group of syndromes and conditions, where the final diagnosis is reached by gradually ruling out individual causes (per exclusionem). In obstetric practice, we most often encounter pregnant women/mothers/new mothers presenting with preeclampsia/HELLP syndrome (haemolysis, elevated liver enzymes, low platelets). This condition, which is otherwise well known to all obstetricians, includes MAHA (dynamic decrease in haemoglobin levels, increase in bilirubin levels, decrease in haptoglobin, presence of schistocytes in peripheral blood smears), periportal liver ischemia (elevated transaminases), and thrombocytopenia due to increased platelet aggregation in the damaged peripheral microcirculation. HELLP syndrome is also classified as TMA, but it should resolve spontaneously within approximately 48–72 h after delivery. If this does not happen, it is very important to consider other causes of TMA, which often pose an even more serious threat to life than HELLP syndrome. Thorough knowledge of differential diagnosis is therefore very important. Every healthcare provider treating pregnant women must therefore have a good understanding of this issue, which is why the authors present it in the form of this recommended procedure.

Keywords:

HELLP syndrome – thrombotic thrombocytopenic purpura – preeclampsia – thrombotic microangiopathy – acute pregnancy-related hepatic steatosis – complement-mediated HUS

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