Obstetric atypical hemolytic uremic syndrome: THE FIRST RUSSIAN EXPERIENCE OF DIAGNOSIS AND TREATMENT
Atypical hemolytic uremic syndrome (AHUs) in 12-31% of cases is associated with pregnancy or delivery, and may determine the prognosis of both mother and child. AIM: Analysis of clinical manifestations, features of course and outcomes of obstetric aHUS. PATIENTS AND METHODS: From 2012 to 2015 were observed 17 patients aged 19 to 38 years, in whom aHUS developed during pregnancy or directly after delivery. RESULTS: The development of aHUS was preceded by a mainly preeclampsia and/or HELLP syndrome and other pregnancy complications, diarrhea. All patients had complete symptom complex of thrombotic microangiopathy (TMA): sharp decrease of hemoglobin level (67,7 ± 15,9 g/l) with signs of microangiopathic hemolysis (increased LDH levels 2737,3 ± 3276,7 U/l, schistocytosis), thrombocytopenia (55,9±30.9 thousand in mcl.), acute kidney injury (hypercreatininemia 447.6 ±of 226.7 mg/DL, oliguria or anuria). Most patients (82%, 14 of 17) had first time mentioned arterial hypertension. In all patients the TMA was of a systemic nature: the signs of damage of liver, central nervous system, lungs had 73% of women (13 of 17), heart - 41% (7 of 17). In the analysis of flow aHUS depending on the stages of pregnancy at the time of the disease development clinical and laboratory parameters were more severe in women with manifestations of AHUs in the third trimester and after delivery. Treatment included plasma exchange and/or plasma infusions (14 of 17, 82%) with following Eculizumab treatment in 7 patients. Maternal mortality was 29%. CONCLUSION. Obstetric aHUS is characterized by the development of multiple organ failure in most patients. Development aHUS preceded by various complement-activating state. It seems that complications of pregnancy, not the pregnancy per se, are triggers of aHUS in women with a genetic predisposition to its development.