Thrombotic Microangiopathy in the Postpartum Period (Literature Review, Clinical Case Report)

Introduction. Thrombotic microangiopathy (TMA) is a heterogeneous group of diseases that, in the presence of endothelial damage, can lead to microvascular thrombosis, secondary platelet consumption, mechanical hemolysis and ischemic end-organs damage. Such triad of symptoms as acute kidney injury (AKI), microangiopathic hemolysis and thrombocytopenia may also accompany some pregnancy-specific conditions (severe preeclampsia/HELLP syndrome (hemolysis, elevated liver enzymes, and low platelet count), acute fatty liver disease of pregnancy (AFLP), and conditions not related to pregnancy, but triggered by it (catastrophic antiphospholipid syndrome (CAPS), lupus flare. The aim of the study. To review the current literature devoted to the problem of thrombotic microangiopathy, to demonstrate on a clinical case a step-by-step differentiated approach to the diagnosis of the type of thrombotic microangiopathy causing acute kidney injury in the postpartum period, and to describe modern methods of treatment of these diseases. Results. The classical types of TMA are thrombotic thrombocytopenic purpura (TTP) and typical hemolytic uremic syndrome (HUS), also known as enterohemorrhagic Shiga toxin-producing Escherichia coli (STEC)-associated HUS (STEC-HUS). Pathophysiologically, all forms of TMA involve complement-mediated endothelial cell damage, which mainly affects the capillary zone of the kidney. Pregnancy can trigger atypical HUS (aHUS) or TTP. The article describes the clinical case of a 37-year-old woman who developed AKI following a complicated delivery. A turn-based differential diagnosis of aHUS was performed. Unwarranted discontinuation of the targeted therapy with Eculisumab led to the development of chronic renal failure Conclusions. Thrombotics microangiopathy are the life-threatening conditions rarely seen in pregnancy making its early recognition difficult. As thrombotics microangiopathy require urgent treatment, plasmapheresis should be started as soon as they are suspected, followed by targeted therapy (Eculisumab, Rituximab) after the confirmation of the diagnosis. This may contribute to reducing maternal morbidity and mortality rates.

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Journal
Lviv clinical bulletin
Published
2024-09-30
DOI
https://doi.org/10.25040/lkv2024.03.084
Primary Topic
Complement system in diseases
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article
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article

Thrombotic Microangiopathy in the Postpartum Period (Literature Review, Clinical Case Report)

H. Kunynets, K.L. Shatylovych, Л. Б. Маркін, S. Serhiichuk et al.
Lviv clinical bulletin
Complement system in diseases
article

Thrombotic Microangiopathy in the Postpartum Period (Literature Review, Clinical Case Report)

H. Kunynets, K.L. Shatylovych, Л. Б. Маркін, S. Serhiichuk, M. Lysyi
article en

Abstract

Introduction. Thrombotic microangiopathy (TMA) is a heterogeneous group of diseases that, in the presence of endothelial damage, can lead to microvascular thrombosis, secondary platelet consumption, mechanical hemolysis and ischemic end-organs damage. Such triad of symptoms as acute kidney injury (AKI), microangiopathic hemolysis and thrombocytopenia may also accompany some pregnancy-specific conditions (severe preeclampsia/HELLP syndrome (hemolysis, elevated liver enzymes, and low platelet count), acute fatty liver disease of pregnancy (AFLP), and conditions not related to pregnancy, but triggered by it (catastrophic antiphospholipid syndrome (CAPS), lupus flare. The aim of the study. To review the current literature devoted to the problem of thrombotic microangiopathy, to demonstrate on a clinical case a step-by-step differentiated approach to the diagnosis of the type of thrombotic microangiopathy causing acute kidney injury in the postpartum period, and to describe modern methods of treatment of these diseases. Results. The classical types of TMA are thrombotic thrombocytopenic purpura (TTP) and typical hemolytic uremic syndrome (HUS), also known as enterohemorrhagic Shiga toxin-producing Escherichia coli (STEC)-associated HUS (STEC-HUS). Pathophysiologically, all forms of TMA involve complement-mediated endothelial cell damage, which mainly affects the capillary zone of the kidney. Pregnancy can trigger atypical HUS (aHUS) or TTP. The article describes the clinical case of a 37-year-old woman who developed AKI following a complicated delivery. A turn-based differential diagnosis of aHUS was performed. Unwarranted discontinuation of the targeted therapy with Eculisumab led to the development of chronic renal failure Conclusions. Thrombotics microangiopathy are the life-threatening conditions rarely seen in pregnancy making its early recognition difficult. As thrombotics microangiopathy require urgent treatment, plasmapheresis should be started as soon as they are suspected, followed by targeted therapy (Eculisumab, Rituximab) after the confirmation of the diagnosis. This may contribute to reducing maternal morbidity and mortality rates.

Lviv clinical bulletin(3 (47))
Danylo Halytsky Lviv National Medical University (UA)
Good health and well-being
Openalex Percentile: Top 75%
Complement system in diseases
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