-FUNCTIONAL/ANTIGEN FIBRINOGEN RATIO IN DIFFERENT CLINICAL SITUATIONS

REMOTTI LUCIA; GROSSO SILVIA HAYDEE; LAZZARI MARÍA ÁNGELA; INGRATTI MARCELO; SÁNCHEZ LUCEROS ANALÍA; BLANCO ALICIA NOEMÍ; VERA MORANDINI MARÍA PAULA; MESCHENGIESER SUSANA SARA

Montpellier

Encuentro; 62nd Annual SSC Meeting of the ISTH; 2016

International Society on Thrombosis and Haemostasis

Background: Inherited disorders of fibrinogen (Fg) are rare and can be divided into quantitative (aandhypo) and qualitative (dysandhypodys).Aims: It was to analyse clinical and laboratory phenotypes of patients with Fg´s defects.Methods: Results of 44 individuals (30 women, 14 men, age range:187year old) with low Fg(M.Clauss) (FgF), were retrospectively analysed. Fg antigen (M.Laurell) (FgAg) and the ratio (R)FgF/FgAg together with other laboratory results were also analysed. Clinical and laboratorybackground, familiar (FH) and personal (PH), was taken into account. Dysfibrinogenemia (dysFg) Rcut off was estimated based on 26 controls.Results: Controls: R (0.691.15)shows normal distribution (pD´AgostinoPearson=0.30) with mean valueof 0.94 (SD=0.14). Rlower limit was 0.67 (mean value2SD;CI 95%).Patients showed FgF (not detectable195mg/dL) under the reference value (200400mg/dL). Resultswere compatible with dysFg (R:0.190.66;FgF:52170mg/dL) in 31.82% (14/44) of patients; the rest(30/44) had R≥0.67 (R:0.711.08;FgF:not detectable195mg/dL). Table shows laboratory and clinical(asymptomatic, bleeding, stroke or obstetric complications) backgrounds; thrombin time (TT)behaviour in patients plasma and mixing tests is also displayed.Other abnormal tests as minor defects of platelet function (9), low FVIII (5, 1/5< 15UI/dL), low plateletcount (2, 1/2< 50x109/L) and possible VWD (1), were detected in some patients with FH and/or PH ofbleeding (6/9 in R< 0.67, and 11/18 in R≥0.67). None of asymptomatic patients or those with stroke orobstetric complications showed additional abnormal tests.R