Atypical hemolytic uremic syndrome in first trimester pregnancy successfully treated with eculizumab

ConclusionEarly recognition of atypical hemolytic uremic syndrome is often difficult as several other conditions also manifest as thrombotic microangiopathy during pregnancy, causing delay in initiating appropriate treatment. Our case suggests that treatment of atypical hemolytic uremic syndrome in early trimester of pregnancy with eculizumab results in good outcome to mother and fetus.
Source: Experimental Hematology and Oncology - Category: Cancer & Oncology Source Type: research

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Complement-mediated thrombotic microangiopathy (TMA), also known as atypical hemolytic uremic syndrome (aHUS) is a rare, hereditary, progressive, life-threatening disorder caused by a disruption in regulation of the alternative pathway of the complement system. Eculizumab, a terminal complement inhibitor, has emerged as a first-line therapy, however data are limited to small case series (Brocklebank et al., 2017). Here, we present a diagnostically challenging case of complement-mediated TMA, who received eculizumab therapy with excellent hematologic response.A 68-year-old female with history of possible Sjogren's syndrome,...
Source: Blood - Category: Hematology Authors: Tags: 311. Disorders of Platelet Number or Function Source Type: research
Publication date: April 2018Source: Pregnancy Hypertension, Volume 12Author(s): M. Gupta, B.B. Feinberg, R.M. BurwickAbstractThrombotic microangiopathy (TMA) disorders are characterized by microangiopathic hemolytic anemia, thrombocytopenia and end-organ injury. In pregnancy and postpartum, TMA is most commonly encountered with HELLP (hemolysis, elevated liver enzymes, low platelet count syndrome) or preeclampsia with severe features, but rarely TMA is due to thrombotic thrombocytopenic purpura (TTP) or atypical hemolytic uremic syndrome (aHUS). Due to overlapping clinical and laboratory features, TTP and aHUS are often mi...
Source: Pregnancy Hypertension: An International Journal of Womens Cardiovascular Health - Category: OBGYN Source Type: research
Publication date: Available online 16 February 2018 Source:Pregnancy Hypertension Author(s): M. Gupta, B.B. Feinberg, R.M. Burwick Thrombotic microangiopathy (TMA) disorders are characterized by microangiopathic hemolytic anemia, thrombocytopenia and end-organ injury. In pregnancy and postpartum, TMA is most commonly encountered with HELLP (hemolysis, elevated liver enzymes, low platelet count syndrome) or preeclampsia with severe features, but rarely TMA is due to thrombotic thrombocytopenic purpura (TTP) or atypical hemolytic uremic syndrome (aHUS). Due to overlapping clinical and laboratory features, TTP and aHUS are o...
Source: Pregnancy Hypertension: An International Journal of Womens Cardiovascular Health - Category: OBGYN Source Type: research
We present one case of HUS caused by adenovirus in a boy of 1 year and 7 months old with severe evolution, which required peritoneal dialysis.Diagnose:Stool sample repeated examination for adenovirus antigen was positive in 2 samples.Intervention:During hospitalization, the patient required 8 peritoneal dialysis sessions.Outcome:The renal function was corrected on discharge, the patient required cardiovascular monitoring 1 month after discharge.Lesson:Although the most common cause that leads to HUS remains STEC, other etiologies like viral ones that may be responsible for severe enteric infection with progression into HUS...
Source: Medicine - Category: Internal Medicine Tags: Research Article: Clinical Case Report Source Type: research
ABSTRACT Thrombotic microangiopathy (TMA) arises in a variety of clinical circumstances with the potential to cause significant dysfunction of the kidneys, brain, gastrointestinal tract and heart. TMA should be considered in all patients with thrombocytopenia and anaemia, with an immediate request to the haematology laboratory to look for red cell fragments on a blood film. Whilst TMA of any aetiology generally demands prompt treatment, this is especially so in thrombotic thrombocytopenic purpura (TTP) and atypical haemolytic uraemic syndrome (aHUS), where organ failure may be precipitous, irreversible and fatal. In all ad...
Source: Nephrology - Category: Urology & Nephrology Authors: Tags: Review Article Source Type: research
We describe how we use these criteria to evaluate and manage pregnant/postpartum women with MAHA and thrombocytopenia. PMID: 26637783 [PubMed - in process]
Source: Hematology ASH Education Program - Category: Hematology Tags: Hematology Am Soc Hematol Educ Program Source Type: research
Conclusions We demonstrate that, even in patients with atypical hemolytic uremic syndrome and prolonged dialysis dependence, recovery of renal function can be seen with eculizumab treatment. We suggest a treatment regime of at least three months prior to evaluation of efficacy.
Source: Journal of Medical Case Reports - Category: Journals (General) Source Type: research
This article aims to review the main diseases included in the differential diagnosis of CAPS in the context of other thrombotic microangiopathies.
Source: Current Rheumatology Reports - Category: Rheumatology Source Type: research
More News: Anemia | Babies | Cancer & Oncology | Dialysis | Genetics | Hematology | Hemodialysis | Hemolytic Uremic Syndrome (HUS) | Hypertension | Pregnancy | Thrombocytopenia | Urology & Nephrology