Pregnancy Associated Atypical Hemolytic Uremic Syndrome: Clinical Case and Literature Review

Pregnancy associated atypical hemolytic uremic syndrome (pa-aHUS) is a rare thrombotic microangiopathy (TMA), fatal in 50-60% of cases. It‘s typical clinical presentation is a triad of microangiopathic hemolytic anaemia, thrombocytopenia and acute progressive renal failure. Plasmapheresis therapy is recommended as a specific treatment. Retrospective analysis of medical documentation of a patient, who was treated for the pa-aHUS in Vilnius University Hospital Santaros Klinikos, was carried out. 44-year old 34th-week pregnant woman was admitted to Vilnius University Hospital Santaros Klinikos with signs of preeclampsia. As preeclampsia progressed, caesarian section was performed. The classical triad of aHUS was observed. The most deviant laboratory values were: haemoglobin 61 g/l, lactate dehydrogenase (LDH) 525 U/I, platelets 52×109/l, urea 22.1 mmol/l, creatinine 437 mmol/l. Dfferential diagnostics between HELLP syndrome, myelodysplastic syndrome, atypical hemolytic uremic syndrome, disseminated intravascular coagulation syndrome and Thrombotic Thrombocytopenic Purpura were carried out. ADAMTS13 activity was 36.4%.Plasmapheresis procedures were initiated, requiring 146 units of fresh frozen plasma for 14 procedures. For the symptomatic treatment, 13 units of red blood cells and 4 units of platelets were used and 6 procedures of hemodialysis were performed. Laboratory signs of hemolysis disappeared and kidney function was restored. Patient was discharged 24 days postpartum. Pa-aHUS requires prompt diagnostics and treatment as well as large expenses of a healthcare institution. A precise differential diagnostics ensures patients the access to the most clinically effective treatment. Timely and complex treatment, including plasmapheresis therapy, significantly improved the outcome of the patient.