Acquired Haemophilia: Clinical and Demographic Data.Results of European Acquired Haemophilia Registry (EACH2).

Abstract

AbstractAbstract 1398Acquired Haemophilia (AH) is an autoimmune disorder, characterized by autoantibodies against the coagulant activity of Factor VIII: C. Is a rare (estimated frequency 1.5 cases per million/year), but severe hemorrhagic condition. EACH2 is a multinational European registry from 90 centres in 11 countries that recruited between 2003–2009, This abstract describes the demographic, clinical and aetiological data recorded in a web database from EACH2 from the largest cohort described in the literature to date. Outcomes of haemostatic therapy, immunosuppression and during pregnancy are described elsewhere. Data were recorded on consecutive patients from each centre. Informed consent was taken as appropriate to each country. Parametric and non-parametric test, were used to analyse the study. There were 501 AH patients included in the registry. The basic demographic data are shown in Table 1.Table 1:Demographic data of the whole group expressed in median, interquartile range (IQR) and rangeAgeFVIII at diagnosis IU/dLInhibitor titre at diagnosis BU/mLHb g/LFemale/Male ratioWhole group n=50174212.89235/266(62–80)(1–5)(4.3–42.5)(7.5–11.3)1:1.313–1040–400.1–28003–15.7In 93 % of the patients the diagnosis was triggered by bleeding in a variety o locations, these were and mainly spontaneous (details will presented in another abstract).). There was no correlation between FVIII level or titre inhibitor and type or severity of bleeding.Aetiology of AH Table 2The delay in diagnosis, stratified in etiologic groups, after detecting abnormal APTT is shown in Table 3Table 3:Time (days) between abnormal APTT and diagnosisAll groupIdiopathicAutoimmune diseaseMalignancyPregnancyEvaluable patients n=49921.62436108Mean110.511Median(0–8)(0–9)(0–5)(0–7)(0–9)IQR0–10910–7960–10910–930–60RangeSurvival analysis (Kaplan-Meyer curves), after 5 years of follow-up was: 71.5% in patients who had eradicated the inhibitor, 47.7% in patients dependent immunosuppressive therapy, and 37.6% in those who had not eradicated the inhibitor In conclusion this prospective multinational European registry (EACH2) includes a large number of patients affected of AH. Idiopathic remains the more frequent aetiological association. Bleeding is the principal clinical manifestation but in a proportion of patients there is a considerable delay in diagnosis. The finding of an abnormal APTT in a bleeding patient with no history of hemorrhagic condition should raise suspicion of AH. Survival in this patient cohort is clearly related to response to immunosuppressive therapy. Disclosures:Marco:Novo Nordisk: Consultancy. Collins:NovoNordisk: Consultancy, Honoraria, The EACH2 registry was funded by Novonordisk; Baxter Healthcare: Consultancy, Honoraria, Membership on an entity's Board of Directors or advisory committees, Research Funding, Speakers Bureau. Knoebl:Novo Nordisk: Consultancy, Membership on an entity's Board of Directors or advisory committees, NovoNordisk fund the EACH2 registry, Research Funding; Baxter Healthcare: Consultancy, Membership on an entity's Board of Directors or advisory committees, Research Funding. Levesque:NovoNordisk:. Baudo:NovoNordisk: Consultancy, Honoraria, NovoNordisk fund the EACH2 registry, Speakers Bureau; Bayer Healthcare: Honoraria, Speakers Bureau. Nemes:Novo Nordisk: Consultancy. Tengborn:Novo Nordisk: Consultancy. Huth-Kuehne:NovoNordisk: Consultancy, Membership on an entity's Board of Directors or advisory committees, NovoNordisk fund the EACH2 registry; Baxter Healthcare: Consultancy, Membership on an entity's Board of Directors or advisory committees. Pellegrini:Novonordisk: Consultancy, Honoraria, Speakers Bureau, The EACH2 registry is funded by Novonordisk.