Atypical hemolytic uremic syndrome in the setting of complement-amplifying conditions: case reports and a review of the evidence for treatment with eculizumab

Asif, Arif; Nayer, Ali; Haas, Christian

doi:10.1007/s40620-016-0357-7

Cited by 70 publications

(67 citation statements)

References 107 publications

(173 reference statements)

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“…Complement activating conditions, such as pregnancy complications (preeclampsia and HELLP), renal transplantation, autoimmune disease, and certain medications have been illustrated as conditions that tend to unmask and precede onset of aHUS in predisposed individuals [10]. Our patient presented with aHUS in the first trimester, a very rare occurrence.…”

Section: Discussionmentioning

confidence: 88%

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

et al. 2017

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Background Atypical hemolytic uremic syndrome is a rare disorder which is known to cause acute thrombotic microangiopathy during pregnancy with poor maternal and fetal outcomes. Atypical hemolytic uremic syndrome is caused mostly by dysregulation of alternative complement pathway secondary to genetic mutations. Most of the cases reported have been in the post-partum period. We report a rare case of a patient who presents with thrombotic microangiopathy in the first trimester of her eleventh pregnancy and was successfully treated with eculizumab.Case presentationA 30-year-old woman presented at 10 weeks of gestation with hypertension, hemolytic anemia, thrombocytopenia, and acute kidney injury, consistent with thrombotic microangiopathy. She was managed initially with daily plasmapheresis. However, her kidney function did not recover, requiring hemodialysis. ADAMTS13 activity was later found to be within normal limit, hence diagnosis of atypical hemolytic uremic syndrome was strongly considered at that time and she was immediately treated with anti-C5 humanized monoclonal antibody (eculizumab). The patient responded well (resolution of thrombotic microangiopathy and recovery of renal function) to eculizumab, with continued remission after discharge and successfully delivered a healthy baby at term without any peripartum complications.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.

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Section: Discussionmentioning

confidence: 88%

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

et al. 2017

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“…Although its efficacy in the treatment of aHUS has been demonstrated in numerous studies, [6][7][8][25][26][27][28] there are only limited reports describing its use in P-aHUS. [29][30][31][32][33][34][35][36][37] Our P-aHUS cohort is the first one to include a significant number of cases treated with eculizumab (n=10). Notably, the eculizumab treatment was successful, with normalization of all hematological parameters and preservation of renal function, in all cases.…”

Section: Discussionmentioning

confidence: 99%

A retrospective study of pregnancy-associated atypical hemolytic uremic syndrome

Huerta

Arjona

Pórtoles

et al. 2018

Kidney International

121

126

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Pregnancy-associated atypical hemolytic uremic syndrome (aHUS) refers to the thrombotic microangiopathy resulting from uncontrolled complement activation during pregnancy or the postpartum period. Pregnancy-associated aHUS is a devastating disease for which there is a limited clinical understanding and treatment experience. Here we report a retrospective study to analyze the clinical and prognostic data of 22 cases of pregnancy-associated aHUS from the Spanish aHUS Registry under different treatments. Sixteen patients presented during the first pregnancy and as many as nine patients required hemodialysis at diagnosis. Identification of inherited complement abnormalities explained nine of the 22 cases, with CFH mutations and CFH to CFHR1 gene conversion events being the most prevalent genetic alterations associated with this disorder (66%). In thirteen of the cases, pregnancy complications were sufficient to trigger a thrombotic microangiopathy in the absence of genetic or acquired complement alterations. The postpartum period was the time with highest risk to develop the disease and the group shows an association of cesarean section with pregnancy-associated aHUS. Seventeen patients underwent plasma treatments with a positive renal response in only three cases. In contrast, ten patients received eculizumab with an excellent renal response in all, independent of carrying or not inherited complement abnormalities. Although the cohort is relatively small, the data suggest that pregnancy-associated aHUS is not different from other types of aHUS and suggest the efficacy of eculizumab treatment over plasma therapies. This study may be useful to improve prognosis in this group of aHUS patients.

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“…However, aHUS has also been known to occur in the absence of these risk factors (36). Eculizumab, an FDA approved humanized monoclonal antibody that targets complement protein C5, has been reported to treat aHUS both in and outside of pregnancy (36,38,60,61). Patients with aHUS have also benefited from plasma exchange which removes and exchanges blood plasma components from and to the circulation.…”

Section: Thrombotic Microangiopathies Associated With Pregnancymentioning

confidence: 99%

Acute Kidney Injury in Pregnancies Complicated With Preeclampsia or HELLP Syndrome

et al. 2020

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Acute kidney injury that occurs during pregnancy or in the post-partum period (PR-AKI) is a serious obstetric complication with risk of significant associated maternal and fetal morbidity and mortality. Recent data indicates that the incidence of PR-AKI is increasing, although accurate calculation is limited by the lack of a uniform diagnostic criteria that is validated in pregnancy. Hypertensive and thrombotic microangiopathic disorders of pregnancy have been identified as major contributors to the burden of PR-AKI. As is now accepted regarding preeclampsia, HELLP syndrome and atypical hemolytic uremic syndrome, it is believed that PR-AKI may have long-term renal, cardiovascular and neurocognitive consequences that persist beyond the post-partum period. Further research regarding PR-AKI could be advanced by the development of a pregnancy-specific validated definition and classification system; and the establishment of refined animal models that would allow researchers to further elucidate the mechanisms and sequelae of the disorder.

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Atypical hemolytic uremic syndrome in the setting of complement-amplifying conditions: case reports and a review of the evidence for treatment with eculizumab

Cited by 70 publications

References 107 publications

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

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

A retrospective study of pregnancy-associated atypical hemolytic uremic syndrome

Acute Kidney Injury in Pregnancies Complicated With Preeclampsia or HELLP Syndrome

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