HELLP Syndrome and Differential Diagnosis with Other Thrombotic Microangiopathies in Pregnancy
Thrombotic microangiopathies (TMAs) comprise a distinct group of diseases with different manifestations that can occur in both pediatric and adult patients. They can be hereditary or acquired, with subtle onset or a rapidly progressive course, and they are particularly known for their morbidity and...
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| Format: | Article |
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MDPI AG
2024-02-01
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| Series: | Diagnostics |
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| Online Access: | https://www.mdpi.com/2075-4418/14/4/352 |
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| author | Stefano Raffaele Giannubilo Daniela Marzioni Giovanni Tossetta Andrea Ciavattini |
| author_facet | Stefano Raffaele Giannubilo Daniela Marzioni Giovanni Tossetta Andrea Ciavattini |
| author_sort | Stefano Raffaele Giannubilo |
| collection | DOAJ |
| description | Thrombotic microangiopathies (TMAs) comprise a distinct group of diseases with different manifestations that can occur in both pediatric and adult patients. They can be hereditary or acquired, with subtle onset or a rapidly progressive course, and they are particularly known for their morbidity and mortality. Pregnancy is a high-risk time for the development of several types of thrombotic microangiopathies. The three major syndromes are hemolysis, elevated liver function tests, and low platelets (HELLP); hemolytic uremic syndrome (HUS); and thrombotic thrombocytopenic purpura (TTP). Because of their rarity, clinical information and therapeutic results related to these conditions are often obtained from case reports, small series, registries, and reviews. The collection of individual observations, the evolution of diagnostic laboratories that have identified autoimmune and/or genetic abnormalities using von Willebrand factor post-secretion processing or genetic–functional alterations in the regulation of alternative complement pathways in some of these TMAs, and, most importantly, the introduction of advanced treatments, have enabled the preservation of affected organs and improved survival rates. Although TMAs may show different etiopathogenesis routes, they all show the presence of pathological lesions, which are characterized by endothelial damage and the formation of thrombi rich in platelets at the microvascular level, as a common denominator, and thrombotic damage to microcirculation pathways induces “mechanical” (microangiopathic) hemolytic anemia, the consumption of platelets, and ischemic organ damage. In this review, we highlight the current knowledge about the diagnosis and management of these complications during pregnancy. |
| first_indexed | 2024-03-07T22:35:47Z |
| format | Article |
| id | doaj.art-b5dbead527be451e922e3636741d9320 |
| institution | Directory Open Access Journal |
| issn | 2075-4418 |
| language | English |
| last_indexed | 2024-03-07T22:35:47Z |
| publishDate | 2024-02-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Diagnostics |
| spelling | doaj.art-b5dbead527be451e922e3636741d93202024-02-23T15:13:39ZengMDPI AGDiagnostics2075-44182024-02-0114435210.3390/diagnostics14040352HELLP Syndrome and Differential Diagnosis with Other Thrombotic Microangiopathies in PregnancyStefano Raffaele Giannubilo0Daniela Marzioni1Giovanni Tossetta2Andrea Ciavattini3Department of Clinical Sciences, Polytechnic University of Marche, Via Corridoni 11, 60123 Ancona, ItalyDepartment of Experimental and Clinical Medicine, Polytechnic University of Marche, Via Tronto 10/a, 60126 Ancona, ItalyDepartment of Experimental and Clinical Medicine, Polytechnic University of Marche, Via Tronto 10/a, 60126 Ancona, ItalyDepartment of Clinical Sciences, Polytechnic University of Marche, Via Corridoni 11, 60123 Ancona, ItalyThrombotic microangiopathies (TMAs) comprise a distinct group of diseases with different manifestations that can occur in both pediatric and adult patients. They can be hereditary or acquired, with subtle onset or a rapidly progressive course, and they are particularly known for their morbidity and mortality. Pregnancy is a high-risk time for the development of several types of thrombotic microangiopathies. The three major syndromes are hemolysis, elevated liver function tests, and low platelets (HELLP); hemolytic uremic syndrome (HUS); and thrombotic thrombocytopenic purpura (TTP). Because of their rarity, clinical information and therapeutic results related to these conditions are often obtained from case reports, small series, registries, and reviews. The collection of individual observations, the evolution of diagnostic laboratories that have identified autoimmune and/or genetic abnormalities using von Willebrand factor post-secretion processing or genetic–functional alterations in the regulation of alternative complement pathways in some of these TMAs, and, most importantly, the introduction of advanced treatments, have enabled the preservation of affected organs and improved survival rates. Although TMAs may show different etiopathogenesis routes, they all show the presence of pathological lesions, which are characterized by endothelial damage and the formation of thrombi rich in platelets at the microvascular level, as a common denominator, and thrombotic damage to microcirculation pathways induces “mechanical” (microangiopathic) hemolytic anemia, the consumption of platelets, and ischemic organ damage. In this review, we highlight the current knowledge about the diagnosis and management of these complications during pregnancy.https://www.mdpi.com/2075-4418/14/4/352HELLPHUSpregnancyTMATTP |
| spellingShingle | Stefano Raffaele Giannubilo Daniela Marzioni Giovanni Tossetta Andrea Ciavattini HELLP Syndrome and Differential Diagnosis with Other Thrombotic Microangiopathies in Pregnancy Diagnostics HELLP HUS pregnancy TMA TTP |
| title | HELLP Syndrome and Differential Diagnosis with Other Thrombotic Microangiopathies in Pregnancy |
| title_full | HELLP Syndrome and Differential Diagnosis with Other Thrombotic Microangiopathies in Pregnancy |
| title_fullStr | HELLP Syndrome and Differential Diagnosis with Other Thrombotic Microangiopathies in Pregnancy |
| title_full_unstemmed | HELLP Syndrome and Differential Diagnosis with Other Thrombotic Microangiopathies in Pregnancy |
| title_short | HELLP Syndrome and Differential Diagnosis with Other Thrombotic Microangiopathies in Pregnancy |
| title_sort | hellp syndrome and differential diagnosis with other thrombotic microangiopathies in pregnancy |
| topic | HELLP HUS pregnancy TMA TTP |
| url | https://www.mdpi.com/2075-4418/14/4/352 |
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