SY10-1. Differential diagnosis of pregnancy-associated thrombotic microangiopathies (TTP, aHUS, and HELLP syndrome)

2021 ◽  
Vol 25 ◽  
pp. e12-e13
Author(s):  
Masanori Matsumoto
Keyword(s):  
Differential Diagnosis ◽  
Hellp Syndrome ◽  
Thrombotic Microangiopathies

scholarly journals Pregnancy onset congenital thrombotic thrombocytopenic purpura (Upshaw-Schulman syndrome) mimicking HELLP syndrome: a case report

2021 ◽  
Vol 29 (3) ◽  
pp. 270-273
Author(s):  
Başak Ergin ◽  
Berna Buse Kobal ◽  
Zeynep Yazıcı ◽  
Ali Hakan Kaya ◽  
Sezin Canbek ◽  
...  
Keyword(s):  
Thrombotic Thrombocytopenic Purpura ◽  
Hellp Syndrome ◽  
Epigastric Pain ◽  
Novel Mutation ◽  
Adamts13 Activity ◽  
Thrombotic Microangiopathies ◽  
Thrombocytopenic Purpura ◽  
Uremic Syndrome ◽  
Congenital Thrombotic Thrombocytopenic Purpura ◽  
Low Platelet Count

Objective Thrombotic thrombocytopenic purpura is a thrombotic microangiopathic condition characterized by hemolytic anemia, thrombocytopenia, neurologic abnormalities, fever and renal dysfunction. Thrombotic microangiopathies such as preeclampsia and HELLP syndrome are pregnancy-specific, whereas others such as thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome are not. In this report, we present a case at which we identified a novel mutation which led to a significant reduction of ADAMTS13 activity. Case(s) A nulliparous pregnant woman of 32-year-old presenting with epigastric pain, hypertension and low platelet count was first suspected of HELLP syndrome, but was diagnosed with congenital TTP after delivery. Conclusion HELLP syndrome co-existed with undiagnosed TTP in this case. We strive to have sufficient awareness in order to distinguish these two pathologies from each other on an antenatal basis, because the causes of the managements are entirely different.

scholarly journals Complement Activation and Thrombotic Microangiopathies

2019 ◽  
Vol 14 (12) ◽  
pp. 1719-1732 ◽  
Author(s):  
Marta Palomo ◽  
Miquel Blasco ◽  
Patricia Molina ◽  
Miquel Lozano ◽  
Manuel Praga ◽  
...  
Keyword(s):  
Endothelial Cells ◽  
Hemolytic Uremic Syndrome ◽  
Acute Phase ◽  
Complement Activation ◽  
Hellp Syndrome ◽  
Atypical Hemolytic Uremic Syndrome ◽  
Malignant Hypertension ◽  
Clinical Relapse ◽  
Thrombotic Microangiopathies ◽  
Uremic Syndrome

Background and objectivesAtypical hemolytic uremic syndrome is a form of thrombotic microangiopathy caused by dysregulation of the alternative complement pathway. There is evidence showing complement activation in other thrombotic microangiopathies. The aim of this study was to evaluate complement activation in different thrombotic microangiopathies and to monitor treatment response.Design, setting, participants, & measurementsComplement activation was assessed by exposing endothelial cells to sera or activated-patient plasma—citrated plasma mixed with a control sera pool (1:1)—to analyze C5b-9 deposits by immunofluorescence. Patients with atypical hemolytic uremic syndrome (n=34) at different stages of the disease, HELLP syndrome (a pregnancy complication characterized by hemolysis, elevated liver enzymes, and low platelet count) or severe preeclampsia (n=10), and malignant hypertension (n=5) were included.ResultsAcute phase atypical hemolytic uremic syndrome–activated plasma induced an increased C5b-9 deposition on endothelial cells. Standard and lower doses of eculizumab inhibited C5b-9 deposition in all patients with atypical hemolytic uremic syndrome, except in two who showed partial remission and clinical relapse. Significant fibrin formation was observed together with C5b-9 deposition. Results obtained using activated-plasma samples were more marked and reproducible than those obtained with sera. C5b-9 deposition was also increased with samples from patients with HELLP (all cases) and preeclampsia (90%) at disease onset. This increase was sustained in those with HELLP after 40 days, and levels normalized in patients with both HELLP and preeclampsia after 6–9 months. Complement activation in those with malignant hypertension was at control levels.ConclusionsThe proposed methodology identifies complement overactivation in patients with atypical hemolytic uremic syndrome at acute phase and in other diseases such as HELLP syndrome and preeclampsia. Moreover, it is sensitive enough to individually assess the efficiency of the C5 inhibition treatment.

scholarly journals Hemolysis, Elevated Liver Enzymes, Low Platelets Syndrome Superimposed on Hemolytic Uremic Syndrome

2017 ◽  
Vol 39 (04) ◽  
pp. 195-198 ◽  
Author(s):  
Inês Martins ◽  
Madalena Conceição ◽  
Paulo Gomes ◽  
Nuno Clode
Keyword(s):  
Hemolytic Uremic Syndrome ◽  
Hellp Syndrome ◽  
Liver Enzymes ◽  
Future Research ◽  
Thrombotic Microangiopathies ◽  
Laboratory Findings ◽  
Reproductive Immunology ◽  
Elevated Liver Enzymes ◽  
Uremic Syndrome ◽  
Nephrotic Range Proteinuria

AbstractA pregnancy complicated by typical hemolytic uremic syndrome (HUS) and hemolysis, elevated liver enzymes, low platelets (HELLP) syndrome is reported. At 20 weeks of gestation, a case of HUS was diagnosed, with Shiga toxin-producing Escherichia coli identified. Plasmapheresis allowed continuation of the pregnancy for 5 weeks. Superimposed preeclampsia and HELLP syndrome were diagnosed after the establishment of nephrotic range proteinuria, hypertension and recurrence of hemolysis. This is a singular case, as it demonstrates that HELLP syndrome can superimpose upon HUS, a fact that can impact future research on reproductive immunology. It also reminds clinicians that the overlapping of clinical and laboratory findings in HELLP syndrome makes the diagnosis of other thrombotic microangiopathies during pregnancy a clinical challenge.

scholarly journals Differential diagnosis of obstetric thrombotic microangiopathy: a review

2021 ◽  
Vol 8 (2) ◽  
Author(s):  
Polina I. Kukina ◽  
Anastasiya V. Moskatlinova ◽  
Irina M. Bogomazova ◽  
Elena V. Timokhina
Keyword(s):  
Differential Diagnosis ◽  
Hemolytic Anemia ◽  
Thrombotic Microangiopathy ◽  
Hellp Syndrome ◽  
Target Organ Damage ◽  
Organ Damage ◽  
Target Organ ◽  
Microangiopathic Hemolytic Anemia ◽  
Atypical Hus ◽  
Obstetric Practice

Thrombotic microangiopathy (TMA) is a clinical and morphological syndrome, which is based on damage of the endothelium. Clinically, TMA is characterized by a triad of symptoms: thrombocytopenia, microangiopathic hemolytic anemia, and target organ damage. In obstetric practice, TMA most often occurs with preeclampsia or HELLP syndrome, atypical HUS, TTP. The review presents the basic differential criteria for the diagnosis of TMA during pregnancy and after childbirth, as well as the management of patients.

scholarly journals A importância dos exames laboratoriais para o diagnóstico diferencial da síndrome de HELLP/ The importance of laboratory testing for differential diagnosis of HELLP syndrome

2020 ◽  
Vol 3 (6) ◽  
pp. 17474-17486
Author(s):  
Maria Regina Prata Pereira Nunes dos Santos ◽  
Annaíc Huyara Alves de Oliveira ◽  
Pâmella Grasielle Vital Dias de Souza
Keyword(s):  
Differential Diagnosis ◽  
Hellp Syndrome ◽  
Laboratory Testing

scholarly journals LDH/AST ratio: a futureresourcefor thrombotic microangiopathies differential diagnosis in pregnancy

2020 ◽  
Vol 11 (1) ◽  
Author(s):  
Lorella Battini ◽  
Mei Federica ◽  
Falchi Nadia ◽  
Tamaraschi Denise ◽  
Bottone Pietro ◽  
...  
Keyword(s):  
Medical Records ◽  
Hellp Syndrome ◽  
Diagnostic Method ◽  
Atypical Hemolytic Uremic Syndrome ◽  
University Hospital ◽  
Therapeutic Management ◽  
Clinical Approach ◽  
Thrombotic Microangiopathies ◽  
Gynecology And Obstetrics ◽  
In Pregnancy

Objective: To evaluate the clinical approach, the diagnostic method and the most appropriate therapeutic management of thrombotic microangiopathies (TMA) in pregnancy, still leading killers in the obstetric area today. Materials and methods: A large review of the international literature and available clinical studies has been carried out in order to define the current state of the art regarding TMA in pregnancy. In the light of this, 9 clinical cases, among 152 TMA cases, of pregnant women hospitalized and who gave birth in the Pisa University Hospital O.O. U.U. Gynecology and Obstetrics 1 and 2 from 2010 to 2019, were identified, analyzed and re-discussed. Results: Analyzing the diagnostic method and the medical records, we made a critical review of these 9 cases, accurately analyzing the diagnoses made. Among these cases, 6 Thrombotic Thrombocytopenic Purpura (TTP), 2 HELLP Syndrome and 1 Atypical Hemolytic Uremic Syndrome (aHUS) were diagnosed during pregnancy. By analyzing the medical records, the diagnostic method and the therapeutic management of these patients, we questioned the diagnoses made. These diagnoses, from our analytical point of view, are partially not corresponding, being 4 cases of TTP and 5 possible cases of aHUS. Conclusion: From the review of our case history, in the Pisa Obstetric clinics, it is possible to find an under diagnosis of the aHUS cases compared to those of TTP and HELLP syndrome, due both to the unavailability of the ADAMTS13 functionality test and to the unused LDH/AST ratio, which in our opinion could represent a future resource in diagnostic approach to thrombotic microangiopathies in pregnancy.

Sign in / Sign up

Export Citation Format

Share Document