ic sC5b-9, showed remnant complement activation on HMEC-1, whereas HI PS demonstrated complement inhibition (fig 2B).IMEX-CONICET-ANM, Buenos Aires, Argentina; 2Hospital Italiano deBuenos Aires, Buenos Aires, Argentina; 3IIHEMA-Academia Nacional de Medicina, Buenos Aires, Argentina Background: Complications of pregnancy in women with aHUS incorporate preeclampsia. This case examine is really a 35-year-old carrier of genetic complement anomalies, with GMNC3 and aHUS diagnosis, at the moment handled with eculizumab (1200 mg/two weeks). Aims: To watch clinical and laboratory parameters with the patient during her second pregnancy. Approaches: Regimen laboratory and complement testing (ELISA) have been performed: (i) total complement exercise (IMTEC, Human Diagnostics), (ii) plasmatic sC5b-9 (BD biosciences) and (iii) C5b-9 formation on endothelial cell line HMEC-1 in presence of human serum (home-made cell-based ELISA). Effects: At 24 weeks (w) of gestation, indicators of hypertensive crisis integrated headaches and vomiting for which magnesium sulfate was administered. CYP3 Activator Storage & Stability Involving 367w, patient suffered nauseas, headaches and blurred vision, and delivered a balanced dwell birth (2.630kg) following caesarean segment. Eculizumab therapy was related with reduced levels of total complement activation (20U/mL) in the course of pre- and post-natal time period. Interestingly, plasmatic sC5b-9 increased significantly among 32w of gestation and initial week puerperium Conclusions: Observations from this aHUS case study recommend that monitoring levels of sC5b-9 through pregnancy could present insight in dose adjustment of anti-C5 therapy. Additional research are necessary to discover other pathological pathway mechanisms associated with C5b-9 complicated formation on cell surface in the course of pregnancy. FIGURE 2 Detection of C5b-9 complex formation on endotelial cell line HMEC-1 using a cell-based ELISA assay. A. Serum from patient for the duration of her pregnancy and postpartum likewise as from pooled human donors (PS) were incubated with HMEC-1 and complement membrane assault complicated was detected employing an anti-human C5b9 antibody. PABSTRACT641 of|INHERITED THROMBOCYTOPENIAS LPB0080|Clinical and Kainate Receptor Antagonist Formulation Biological Assessment in the 2nd Pedigree Affected with X-linked GATA-1 Associated Thrombocytopenia and Blood Group Lutheran NullConclusions: We report the second pedigree with thrombocytopenia connected for the GATA-1 p.X414R variant, consolidating the romance of this uncommon variant with Lu null phenotype.PB0866|The Copenhagen Founder Variant GP1BA c.58TG is usually a. Rodriguez-Al one; V. Palma-Barqueros2; N. Roll -Sim one; N. Revilla3; N. Bohdan2; J. Padilla2; A. Zamora-C ovas2; A. Mar Qu ez4; A. S chez-Fuentes2; J.R. Gonz ez-Porras4; V. Vicente2; J. Cuesta1; M.L. Lozano2; J.M. Bastida4,5; R. Pozo2,Causal of Monoallelic Bernard-Soulier Syndrome E. Lein 1; N. Broens1; A.O. Rasmussen2; M. Gabrielaite2; S. Rosthoej3; E. Zetterberg4; S.R. Ostrowski5; M. RossingHospital Virgen de la Salud, Complejo Hospitalario de Toledo,Department of Haematology, Rigshospital University Hospital,Toledo, Spain; 2Servicio de Hematolog y Oncolog M ica, Hospital Universitario Morales Meseguer, Centro Regional de Hemodonaci , Universidad de Murcia, IMIB-Arrixaca, CIBERER-U765, Murcia, Spain;Copenhagen, Denmark; 2Genomic Medication, Rigshospitalet University Hospital, Copenhagen, Denmark; 3Department of Pediatrics, Aalborg University Hospital, Aalborg, Denmark; 4Clinical Coagulation Investigate Unit, Department of Translational Medication, Lund University, Malm Sweden; 5Department of