MP158ATYPICAL HEMOLYTIC-UREMIC SYNDROME IN PREGNANCY AND POSTPARTUM: COURSE AND OUTCOMES

Abstract

Introduction and Aims: Atypical hemolytic-uremic syndrome (aHUS) occurs due to uncontrolled complement activation. In 12-31 % of cases, aHUS is associated with pregnancy or delivery, and may determine the prognosis of both mother and child. Aims: Analysis of clinical manifestations, course and outcomes of obstetric aHUS Methods: From 2012 to 2015 we observed 18 patients aged 19 - 38 years with aHUS, occurred during pregnancy or postpartum period. Before the present pregnancyall women were healthy. Depending on the period of development of aHUS all patients were divided into 2 groups: 1 gr. - 13 patients (72%) with the development of aHUS after delivery, 2 gr. - 5 patients (28%) with aHUS manifestation in the second trimester of pregnancy. Results: Preeclampsia and/or HELLP syndrome in combination with obstetric complications (bleeding, manual separation of the placenta, Caesarean delivery, etc.) preceded aHUS manifestation in all patients in the 1st group and diarrhea preceded aHUS in 60% (3 of 5) patients in 2 group. All pts (100%) had complete symptom complex TMA: microangiopathic hemolysis (hemoglobin level to 66.3 ± 16.6 g/l), LDH level 2929,2 ± 3269,9 U/l, schistocity), thrombocytopenia (52,9±35,0x103μL.) and AKI (acute kidney impairment) (creatinine level 463,06 ±222,8mg/DL, oliguria or anuria). The majority of pts (89%, 16 of 18) had increased blood pressure. All women had extrarenal manifestations with sings of liver damage, central nervous system, the lungs in 78% women (14 of 18), heart damage in 39% (7 of 18) pts, pancreas - 22% (3 of 18) pts, gastroenteric ulceration with bleeding-16,6% (3 of 18) pts, ulcerative lesion of bladder- 1 pts. 17 out of 18 pts (94%) had more than one extrarenal damage, 12 of them - more than 3 lesions. The patients in 1st group had more severe course compared with the 2nd group: Hb level 62 [32;102]g/l vs 77 [67;89]g/l (p=0.03), LDH level 2844[702;12484]U/l vs 843 [520;2475]U/l (p=0.05), platelet count 31[11;125]x103μL vs 96[56;100]x103μL (p=0.009), bilirubin level 82[5;347]mg/dL vs 15[14;22]mg/dL (p=0,10). Treatment included plasma exchange and/or plasma infusions (14 of 19, 74%) with following Eculizumab treatment in 8 patients. Favorable outcome with complete normalization of lab parameters and clinical improvement with normalization kidney function was achieved in 9 women. In 2 patients chronic kidney disease (CKD) stages 3 to 4 has occurred as outcome of AKI. Maternal mortality was 26% (5 of 18 pts). Conclusions: Our results indicate that pregnancy-associated aHUS is characterized by the development of multiorgan failure. We suppose the preeclampsia and/or HELLP syndrome can be early aHUS manifestation in women with a genetic predisposition. In these patients pregnancy complications, but not pregnancy per se, can induce the development of aHUS.