Severe HELLP syndrome masquerading as thrombocytopenic thrombotic purpura: a case report

Mousseaux, Cyril; Joly, Bérangère S.; Mohamadou, Inna; Arrestier, Romain; Hertig, Alexandre; Rafat, Cédric

doi:10.1186/s12882-020-01865-y

Cited by 5 publications

(8 citation statements)

References 15 publications

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“…Moreover, HELLP syndrome or preeclampsia should also be considered in pregnant women with a personal history of hereditary or acquired TTP. It should also be noted that the coexistence of TTP and PE/HELLP syndrome should not be excluded from the beginning [ 42 , 43 , 44 , 45 , 46 ]. Otherwise, PE/HELLP are thought to be more frequent in patients who have already recovered from TTP, or even induce the development of TTP in predisposed pregnant women [ 42 , 45 , 46 ].…”

Section: Discussionmentioning

confidence: 99%

“…It should also be noted that the coexistence of TTP and PE/HELLP syndrome should not be excluded from the beginning [ 42 , 43 , 44 , 45 , 46 ]. Otherwise, PE/HELLP are thought to be more frequent in patients who have already recovered from TTP, or even induce the development of TTP in predisposed pregnant women [ 42 , 45 , 46 ]. Although delivery is not mandatory in TTP, it is required when TTP is combined with preeclampsia, even at a gestational age before 34 weeks [ 47 ].…”

Section: Discussionmentioning

confidence: 99%

“…Consequently, even if a deficient level of ADAMTS13 activity is highly diagnostic of TTP, a more average one cannot exclude the possibility of HELLP syndrome/preeclampsia. Nevertheless, it is believed that a complete absence of ADAMTS13 activity is not compatible with the diagnosis of HELLP syndrome/preeclampsia [ 46 ]. The differential diagnosis in patients with average levels of ADAMTS13 activity is usually based on clinical parameters, and further research is needed to establish the use of new and more reliable biomarkers.…”

Section: Discussionmentioning

confidence: 99%

“…Table 4 presents data from several case reports, collected from our literature research process, indicating the difficulties in setting the definite diagnosis. It should be noted that there are many cases in which the final diagnosis remains vague, or the authors attribute the unsuccessful initial management to a possible coexistence of HELLP/PE and TTP from the beginning [ 43 , 44 , 45 , 46 , 47 , 48 ].…”

Section: Discussionmentioning

confidence: 99%

“…In case there is no resolution or even worse, there is a deterioration of the clinical manifestations or the laboratory parameters (persistent anemia, neurological symptoms, further thrombocytopenia) in the first days postpartum, TTP is the most probable diagnosis and plasmapheresis should be performed without any further delay. In the literature, there are several case reports in which TTP was diagnosed postpartum because of deteriorating clinical and laboratory parameters after delivery [ 42 , 43 , 44 , 45 , 46 , 47 , 48 ].…”

Section: Discussionmentioning

confidence: 99%

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Molecular Advances in Preeclampsia and HELLP Syndrome

Gardikioti

Venou

Gavriilaki

et al. 2022

IJMS

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Preeclampsia (PE) constitutes one of the principal reasons for maternal and perinatal morbidity and mortality worldwide. The circumstance typically implicates formerly healthful normotensive women, after 20 weeks of gestation, typically withinside the third trimester, without regarded threat elements or past deliveries. PE can be further complicated with hemolysis and thrombocytopenia, leading to the emergence of HELLP syndrome (Hemolysis, Elevated Liver enzymes, Low platelets). Both conditions are classified as hypertensive diseases of pregnancy (HDP), and their pathogenesis has been linked to an excessive maternal inflammatory response, accompanied by enhanced endothelial activation. Several studies have found that in pregnancies affected by PE/HELLP, von Willebrand factor (vWF) antigen levels (vWF:Ag) are significantly elevated, while its cleaving protease (ADAMTS-13, A Disintegrin-like and Metalloprotease with Thrombospondin type 1 motif, member 13) activity is normal to decreased. Furthermore, the higher urine excretion of the terminal complement complex C5b-9, as well as its greater deposition in the placental surface in preeclamptic women, imply that the utero-placental unit’s distinctive deficits are intimately tied to disproportionate complement activation. The goal of this updated evaluation is to provide the most up-to-date molecular advances in the pathophysiology of PE/HELLP syndromes. Recent medical data on vWF:Ag levels in patients with PE, ADAMTS-13, and dysregulation of the complement system, are highlighted and evaluated. Furthermore, we discuss the relationship between those entities and the progression of the disease, as well as their significance in the diagnostic process. Finally, considering the difficulties in analyzing and controlling those symptoms in pregnant women, we can provide a current diagnostic and therapeutic algorithm.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

See 3 more Smart Citations

Molecular Advances in Preeclampsia and HELLP Syndrome

Gardikioti

Venou

Gavriilaki

et al. 2022

IJMS

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Thrombotic thrombocytopenic purpura and severe preeclampsia: a clinical overlap during pregnancy and a possible coexistence

Dap

Romiti²,

Dolenc³

et al. 2022

Journal of Gynecology Obstetrics and Human Reproduction

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Do Case Reports and Case Series Generate Clinical Discoveries About Preeclampsia? A Systematic Review

Janoudi¹,

Uzun²,

Boyd³

et al. 2023

IJWH

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Background Preeclampsia is a leading cause of maternal and perinatal mortality and morbidity. The management of preeclampsia has not changed much in more than two decades, and its aetiology is still not fully understood. Case reports and case series have traditionally been used to communicate new knowledge about existing conditions. Whether this is true for preeclampsia is not known. Objective To determine whether recent case reports or case series have generated new knowledge and clinical discoveries about preeclampsia. Methods A detailed search strategy was developed in consultation with a medical librarian. Two bibliographic databases were searched through Ovid: Embase and MEDLINE. We selected case reports or case series published between 2015 and 2020, comprising pregnant persons diagnosed with hypertensive disorders of pregnancy, including preeclampsia. Two reviewers independently screened all publications. One reviewer extracted data from included studies, while another conducted a quality check of extracted data. We developed a codebook to guide our data extraction and outcomes assessment. The quality of each report was determined based on Joanna Briggs Institute (JBI) critical appraisal checklist for case reports and case series. Results We included 104 case reports and three case series, together comprising 118 pregnancies. A severe presentation or complication of preeclampsia was reported in 81% of pregnancies, and 84% had a positive maternal outcome, free of death or persistent complications. Only 8% of the case reports were deemed to be of high quality, and 53.8% of moderate quality; none of the case series were of high quality. A total of 26 of the 107 publications (24.3%) included a novel clinical discovery as a central theme. Conclusion Over two-thirds of recent case reports and case series about preeclampsia do not appear to present new knowledge or discoveries about preeclampsia, and most are of low quality.

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Severe HELLP syndrome masquerading as thrombocytopenic thrombotic purpura: a case report

Cited by 5 publications

References 15 publications

Molecular Advances in Preeclampsia and HELLP Syndrome

Molecular Advances in Preeclampsia and HELLP Syndrome

Thrombotic thrombocytopenic purpura and severe preeclampsia: a clinical overlap during pregnancy and a possible coexistence

Do Case Reports and Case Series Generate Clinical Discoveries About Preeclampsia? A Systematic Review

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