Pregnancy-associated atypical haemolytic uraemic syndrome in the postpartum period: a case report and review of the literature

Egbor, Michael; Johnson, Antoinette; Harris, Fiona; Makanjoula, Dorothy; Shehata, Hassan

doi:10.1258/om.2011.100059

Cited by 9 publications

(9 citation statements)

References 19 publications

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“…syndrome (13,14), and in association with certain drugs (15), tumors (16), autoimmune diseases (17,18), malignant hypertension (19), and transplant-associated TMAs (20,21).…”

Section: Introductionmentioning

confidence: 99%

Complement Activation and Thrombotic Microangiopathies

Palomo¹,

Blasco²,

Molina³

et al. 2019

CJASN

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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.

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“…syndrome (13,14), and in association with certain drugs (15), tumors (16), autoimmune diseases (17,18), malignant hypertension (19), and transplant-associated TMAs (20,21).…”

Section: Introductionmentioning

confidence: 99%

Complement Activation and Thrombotic Microangiopathies

Palomo¹,

Blasco²,

Molina³

et al. 2019

CJASN

View full text Add to dashboard Cite

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“…The incidence of aHUS among all pregnancies is reported to be only 1 in 25,000, and recent studies have clearly demonstrated that P-aHUS is a commonly encountered subtype of aHUS, accounting for nearly 20% of all women with this situation. [10][11][12] Thus, pregnancy can be considered an important factor for aHUS development. However, there appears to be no definite period for the onset of aHUS, which could appear at different times, including midpregnancy, peripartum or postpartum.…”

Section: Discussionmentioning

confidence: 99%

Successful treatment with eculizumab in a patient with pregnancy-associated atypical hemolytic uremic syndrome

et al. 2021

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Pregnancy‐associated atypical haemolytic uraemic syndrome is a rare and potentially lethal complement-mediated disorder. It can mimic preeclampsia, gestational hypertension, thrombotic thrombocytopenic purpura and hemolysis, elevated liver enzymes and low platelets syndrome. Thus, it can be hard to distinguish pregnancy‐associated atypical haemolytic uraemic syndrome from other causes in peri/post-partum women presenting with features of microangiopathic haemolytic anemia, thrombocytopenia and acute kidney injury. We present a case of a 35-year-old woman in her third pregnancy at 32 weeks’ gestation who underwent caesarean section due to fetal distress. She developed severe renal impairment, thrombocytopenia and neurologic symptoms within 24 hours after delivery. A diagnosis of pregnancy‐associated atypical haemolytic uraemic syndrome was provided, and treatment with plasma therapy followed by eculizumab was initiated. A rapid improvement of both clinical and laboratory parameters was observed. This case demonstrates the significance of early initiation of anti-complement therapy to prevent irreversible renal damage and possible death in women with pregnancy‐associated atypical haemolytic uraemic syndrome.

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“…1,15,22 The issue, however, is differentiating P-aHUS from HELLP syndrome as demonstrated in our case and in the literature. [4][5][6]15,23 The initial diagnosis in our case was HELLP syndrome; however, the rapid deterioration in renal function with new microscopic haematuria raised the strong clinical suspicion of aHUS. The difference between aHUS and HELLP syndrome is often subtle, if present, with P-aHUS demonstrating a more severe form of acute kidney injury (AKI) with TMA and HELLP syndrome demonstrating more hepatic ischemia.…”

Section: Discussionmentioning

confidence: 99%

“…3 The clinical and biochemical features of P-aHUS overlap with preeclampsia and HELLP syndrome, hence, often resulting in a clinical conundrum. [4][5][6] The introduction of eculizumab has revolutionized the way clinicians manage aHUS with demonstrable improvement in morbidity. 7,8 Our case highlights the importance of early recognition and management of P-aHUS.…”

Section: Introductionmentioning

confidence: 99%

Pregnancy-induced atypical haemolytic uremic syndrome: A new era with eculizumab

2017

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Pregnancy is a well-recognised trigger of atypical haemolytic syndrome (P-aHUS) and often occurs in the post-partum period. Similar to atypical haemolytic uremic syndrome, it carries a poor prognosis with high morbidity particularly in the form of renal failure. Early recognition and intervention is crucial in its management particularly with the recent availability of eculizumab, a humanized monoclonal antibody to complement component C5, which has demonstrated drastic improvement in prognosis. The issue, however, is arriving at a timely diagnosis given the considerable amount of overlap in the clinical and biochemical manifestation of P-aHUS, HELLP syndrome (haemolysis, elevated liver enzyme and low platelet count) and other hypertensive disorders of pregnancy. We present a case report and literature review that highlights the clinical conundrum of arriving at the diagnosis. We also highlight the importance of early management of P-aHUS with eculizumab and its impact on improving morbidity.

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Pregnancy-associated atypical haemolytic uraemic syndrome in the postpartum period: a case report and review of the literature

Cited by 9 publications

References 19 publications

Complement Activation and Thrombotic Microangiopathies

Complement Activation and Thrombotic Microangiopathies

Successful treatment with eculizumab in a patient with pregnancy-associated atypical hemolytic uremic syndrome

Pregnancy-induced atypical haemolytic uremic syndrome: A new era with eculizumab

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