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Introduction Acquired hemophilia (AH) is a rare disorder due to the production of autoantibodies against factor VIII or IX procoagulant function. The development of these autoantibodies results in hemorrhagic symptoms in patients without previous coagulopathy. The typical clinical manifestations of the acquired hemophilia are: extensive cutaneous purpura and internal hemorrhages while hemarthrosis are not common. Acquired hemophilia is less frequent than the inherited form of the disease, with an estimated incidence of 2 to 4 cases/million inhabitants/year, value that increases with age, but is extremely infrequent in children (0.045 cases/million/year). It affects all ethnic groups and has a biphasic distribution with a peak incidence in subjects between 20 and 30 years and another peak between 60 and 80 years. Objective To report a case of AH in a 2-year-old child. Clinical data: Hematoma in the left iliac flank, forearm and legs.Personal history: Otherwise healthy 2-year-old boy with a twin brother and both parents without a history of bleeding disorders. Prior to the onset of bruising, the patient suffered an important trauma on one foot without bleeding complications. A short time after this episode he presented a hematoma in the gluteus that was interpreted as result of a slight trauma due to a fall. Subsequently, he presented multiple spontaneous hematomas that motivate the consultation. • Laboratory upon admission: Hemoglobin: 12.8g/dL; PT: 11" (Control: 11"); APTT:>180" (Control: 30"). Plt: 543000/mm3 • Clinical evolution: With the suspicion of bleeding reactivation, he was referred to the Rosario Hemophilia Foundation to be evaluated. Reactivation of bleeding was ruled out and imaging had shown subcutaneous bleeding without muscle involvement. The presence of an inhibitor to Factor VIII was detected. The patient was commenced on Prednisolone 1mg/kg/day to eradicate the acquired antibody. • Laboratory Results: Hb: 12.6 g/dL, Plt: 575000/mm3, PT: 12" (11") (Innovin), APTT: 76" (30") (Actin FSL) which does not normalize after addition of normal plasma and potentiate when was incubated 2 hours at 37°C, TT: 14"(14") (Trombin), FVIII Conclusions AH is a rare autoimmune disease first described in 1940 and is extremely infrequent in children younger than 16 years. Although it is usually associated with other autoimmune disorders, in our patient, we have not found other autoimmunity markers. We should suspect this pathology in all patients who begin with cutaneo-mucosal bruising without a previous history of bleeding, with prolonged APTT that does not correct with the addition of normal plasma and whose prolongation potentiates after incubating the mixture for 2 hours at 37˚C. These results are usually associated with PT and normal platelets count. The FVIII inhibitor can be quantified by the Bethesda-Nijmegen method. Although the title of the Inhibitor does not correlate well with the clinical severity of the patient, its value is useful in monitoring the immunosuppressive therapeutic response. Finally, although AH is unusual in pediatrics, it must be taken into account among the differential diagnoses. Disclosures No relevant conflicts of interest to declare.

Abstract 5093 Introduction: G6PD deficiency is a frequent worldwide enzymopathy, with sex-linked inheritance, the gene that codifies the enzyme localizes at the X chromosome. Males are hemizygous (Hem) for disease, while women are heterozygous (Het), i.e. carriers, and very rarely homozygous, or double heterozygous and consequently, develop disease. There are over 120 deficient molecular variants observed in Afro-Americans and in the malaria endemic region of the Mediterranean Basin. In previous studies in Argentina we have reported a low prevalence, 0,2-0,3 %, although the predominant molecular variants are unknown. Most of deficiencies are clinically detected when the patient is submitted to oxidative stress produced by infections, therapeutic drugs, or Vicia fava ingestion, develops an acute hemolytic anemia (AHA) of variable severity. Materials and Methods: Two male patients and their families were studied. 1) A1: 45y aged male, with AHA after bean ingestion, as well as the following relatives: mother (M1); sister (S1); three nephews (N1, N2, N3) and a niece (N4) without positive AHA history. 2) A2: 55y aged male, with two AHA episodes in childhood and adolescence, one of them associated with an infection, and the mother (M2). The evaluation clinical and hematological of the patients were normal Laboratory assays: Hemogram, Hemoglobin (Hb) (Coulter AcT10); Reticulocytes (Ret); Brewer test (B.T.); Heinz bodies (HB); Cytochemical method modified (Gurbuz, N; et al): NV: 86,8 % positive cells (CYT); Enzymatic activity (EA) Kinetic Method (Beutler-OMS): NV: 8.0±1.6 G6PD/gHb IU and Polymerase Chain Reaction with Enzyme Restriction (PCR-ER) for characterization of different variants: G6PDMed(563G□T), G6PD A376(A□G), G6PD A376(A□G) 202(G□A); G6PD A 376(A□G) 680 (G□T) y G6PD A 376(A□G) 968 (T□C). Results: Conclusions: The EA confirmed the deficit of probands and nephews N1 and N3 without previous AHA history. The heterozygous character of females was not defined by this method but PCR-ER and CYT mod. supported the obliged carriers heterozygosis and the potential ones, demonstrating that CYT is more sensitive for carrier detection than other screening techniques or EA. The deficient phenotype was correlated with the detected variants, since the Mediterranean G6PD: G6PD Med(563G□T) (Type II, OMS) and G6PD A376(A□G) 202(G□A) (Type III, OMS) are associated with AHA induced by infections or Vicia Fava ingestion. The finding in Argentina of these two variants, that are frequent in the Mediterranean region, it is in accordance with the fact that our population has mainly Italian, Spanish (family 1) and Jewish (family 2) ancestors. The detection of deficient without hemolysis, as well as carriers is of the utmost importance in genetic counseling. Disclosures: No relevant conflicts of interest to declare.