Pregnancy-associated hemolytic uremic syndrome.

Pregnancy-associated hemolytic uremic syndrome (P-aHUS) is not an uncommon condition. It is considered a medical emergency that is associated with a high risk of mortality and serious morbidity. End-stage renal disease as a consequence of P-aHUS occurs in >50% of the patients if left untreated; the majority of identified cases (79%) are during the postpartum period. Its mechanism of action is related mainly to the disturbance in the activation of the complement alternative pathway, leading to damage of the microvascular endothelium. The clinical picture of P-aHUS mimics several conditions occurring during post-partum thrombotic microangiopathy, for example, severe pre-eclampsia, hemolysis, elevated liver enzymes, and low platelet count, thrombotic-thrombocytopenic purpura, and acute fatty liver of pregnancy. Genetic analysis of known genetic mutations together with the analysis of anti-CFH antibodies might confirm the diagnosis of aHUS in the post-partum period. The absence of causative genetic mutations does not always exclude a diagnosis of aHUS, since 40% of patients show no known genetic abnormalities. The mainstay of management is supportive care and immediate initiation of plasmapheresis. Eculizumab has been proved to be both safe and effective in inducing and maintaining remission in P-aHUS and it is recommended to be started as soon as the diagnosis is established.

Three months interval therapy of Eculizumab in a patient with atypical hemolytic uremic syndrome with hybrid CFHR1/CFH gene.

Pregnancy-associated atypical hemolytic uremic syndrome (P-aHUS) is a rare condition. It is characterized by very high maternal mortality and morbidity. Most cases of P-aHUS (79%) manifest in the postpartum period; this is probably due to the complement's involvement in aHUS pathogenesis. Eculizumab is approved for aHUS treatment, but its use is limited due to cost, unknown duration of treatment, and vague dose intervals to keep patients in remission. In this case report, we present a 26-year-old female with P-aHUS with hybrid CFHR1/CFH gene. Eculizumab was initiated after 5 weeks of being on hemodialysis and plasmapheresis sessions. Full remission successfully achieved after 6th dose of Eculizumab, within 13 weeks of onset of aHUS. Due to financial issues and inability to financially cover the cost, Eculizumab was set in hold. Within 6 months, she suffered recurrence of the disease and Eculizumab was re-instated. After re-inducing full remission, the patient was switched to Eculizumab every 3 months instead of the recommended manufacture dose interval of every 2 weeks. We followed this patient for 3 years and she continued to be in remission based on clinical and laboratory data. In conclusion, achievement of successful and maintenance of remission of P-aHUS in this patient who had limited access to Eculizumab raise the attention of the efficacy of Eculizumab at longer time intervals. However, it is time to consider conducting a long-term study to learn about the safety and efficacy of this approach, which may have a major financial advantage for patients.