MP266HELLP-SYNDROME A LINK TO AHUS: LABORATORY FEATURES OF PREECLAMPSIA (PE), HELLP-SYNDROME AND PREGNANCY-ASSOCIATED ATYPICAL HEMOLYTIC UREMIC SYNDROME (P-AHUS)

Abstract

INTRODUCTION AND AIMS: To assess and compare the severity of hematological, renal and liver manifestation, the blood PLGF and sFlt1 levels in patinets with HELLP-syndrome, severe PE and aHUS, and evaluate the association markers with the severity of clinical manifestations. METHODS: Women with classic HELLP, P-aHUS, PE with severe features, and with normal pregnancies were recruited for the retrospective study from September 1, 2013 to December 31, 2016 (treated or consulted in our center via telemedicine). Severe PE diagnosed in accordance with the WHO criteria of 2008. HELLP diagnosed in accordance with Tenessee criteria (laboratory parameters normalized beyond 48-72 hours after delivery without plasma).P-aHUS- rapidly progressive after delivery or onset MAHAT without ADAMTS 13 deficiency. RESULTS: Gr3 had a poor outcome and most severe course: 26/31 gr.3 -had a PE before delivery (9/31 developed HELLP),10/31 had anthenathal child death, 9/31 with signs of severe PE or ACD were urgency delivered, 7/31 had hemorrhage 1000-2000ml. 6/31 had extirpation of uterus. 9/31had signs of heart damage,18/31, a variety of neurological manifestations, 16/31 ARDS, 10/31 renal replacement therapy. However, in addition to PE they had a lot other complement amplifying triggers such as antenatal death of the fetus, placenta abruption, bleeding etc. Patients gr 2 characterized by less severe course: 5/29 had ACD, 4/29 hemorrhage (1000-2000ml), 5/29 signs of heart damage,16/29, a variety of neurological manifestations, 2/29 ARDS, 2/29 renal replacement therapy, at 2/29 VTE. CONCLUSIONS: P-aHUS and HELLP is life-threatening disorders suggests unique predisposing features and responses to injury. HELLP-syndrome may be one of the stages of PaHUS and may developed without PE. We suppose the PE and HELLP are various clinical conditions with different outcomes. Based on clinical findings in PaHUS, we propose a similar mechanism for a pathogenetic role of complement in HELLP. PE is only trigger or complement-activating condition for development HELLP-syndrome. Depending on the triggering stimuli and vascular bed involved, aHUS or the HELLP syndrome may develop. There were more severe clinical manifestations of renal impairment in all pts with HELLP and PaHUS as compared to women with PE and control gr. The sFlt-1 level was significantly higher in pts with PE as compared with HELLP and HELLP-onset aHUS. Less increased ratio of sFlt-1 / PlGF in gr.1 may confirm that PE is only complement amplifying factor to HELLPdevelopment. (Table Presented) .