6533b86dfe1ef96bd12ca128
RESEARCH PRODUCT
Demographic and clinical data in acquired hemophilia A
P KnoeblP MarcoF BaudoP CollinsA Huth KühneL NemesF PellegriniL TengbornH LévesqueAspoeck GHeistinger MKnöbl PMakipernaa AAndré HAouba ABellucci SBeurrier PBorg JyDarnige LDevignes JD'oiron RGautier PGay VGirault SGruel YGuerin VHézard NKhellaf MKoenig MLévesque HLifermann FMarlu RPeynet JQuéméneur TRothschild CSchleinitz NSigaud MTrouillier SVoisin SGiebl AHolstein KHuth Kühne ALoreth RmSteigerwald UTiede ATheodossiades GNemes LRadvanyi GSchlammadinger ABarillari GPasca SBaudo FCaimi TContino LDi Minno GCerbone AmDi Minno DD'incà MFalanga AMaggioni ALerede TFranchini MGaidano GDe Paoli LGamba GGhirardi RGirotto MTasca DGrandone ETiscia GImberti DIorio ALandolfi RDi Gennaro LNovarese LMariani GLapecorella MMarietta MPedrazzi PMazzucconi MgSantoro CMorfini MLinari SMoratelli SPaolini RPiseddu GPoggio REnrico Maria PoglianiCarpenedo MRemiddi CSantagostino ESantoro RPapaleo GSchinco PBorchiellini AScortechini ArSiragusa SSottilotta GSquizzato ASartori RTagariello GTagliaferri ArDi Perna CRivolta GfTesta SPaoletti OToschi VZanon EBrandolini BHamulyák KKamphuisen PLaros Van Gorkom BLeebeek FwMarten NNovakova ISchutgens RVan Der Linden PwVan Esser JVan Der Meer JYpma PCampos MAguilar CAltisent CBermejo NDel Campo RFerreiro Argüelles MGonzález Boullosa RGutiérrez Pimentel MjJiménez Yuste VJose Felix LPascual MMingot MePerez Garrido RPerez Gonzale NzPrieto Garcia MRodriguez Huerta AmSedano CTolosa Munoz ABaghaei FTengborn LBoehlen FKorte WChowdary PCollins PEvans GPavord SRangarajan SWilde J.subject
RegistrieMalePediatricsdiagnosismedicine.medical_treatmentHemostatic TechniqueKaplan-Meier EstimateregistryTHERAPYSettore MED/15 - Malattie Del SangueImmunosuppressive AgentIMMUNOADSORPTIONRisk FactorsPregnancy80 and overProspective StudiesRegistriesProspective cohort studyhealth care economics and organizationsAged 80 and overtreatmentImmunosuppressionHematologyMiddle AgedFACTOR-VIII INHIBITORAcquired hemophilia; Demographics; Diagnosis; Outcome; Registry; Treatment; Aged; Aged 80 and over; Autoantibodies; Chi-Square Distribution; Europe; Factor VIII; Female; Hemostatic Techniques; Humans; Immunosuppressive Agents; Kaplan-Meier Estimate; Male; Middle Aged; Pregnancy; Prospective Studies; Registries; Risk Assessment; Risk Factors; Treatment Outcome; Hemophilia A; HemorrhageAutoantibodieEuropeTreatment Outcomeacquired hemophilia; demographics; diagnosis; outcome; registry; treatmentoutcomeINTRAVENOUS GAMMA-GLOBULINFemaleacquired hemophiliaImmunosuppressive AgentsHumanmedicine.medical_specialtyHemorrhageHemophilia AMalignancyRisk Assessmenthemophilia registrydemographicsmedicineHumansMETAANALYSISAutoantibodiesAgedAutoimmune diseasePregnancyChi-Square DistributionFactor VIIIHemostatic Techniquesbusiness.industryRisk FactorAutoantibodymedicine.diseaseSurgeryProspective StudieHemostasisbusinessChi-squared distributiondescription
Summary. Background: Acquired hemophilia A (AHA) is a rare autoimmune disease caused by autoantibodies against coagulation factor VIII and characterized by spontaneous hemorrhage in patients with no previous family or personal history of bleeding. Although data on several AHA cohorts have been collected, limited information is available on the optimal management of AHA. Objectives: The European Acquired Hemophilia Registry (EACH2) was established to generate a prospective, large-scale, pan-European database on demographics, diagnosis, underlying disorders, bleeding characteristics, treatment and outcome of AHA patients. Results: Five hundred and one (266 male, 235 female) patients from 117 centers and 13 European countries were included in the registry between 2003 and 2008. In 467 cases, hemostasis investigations and AHA diagnosis were triggered by a bleeding event. At diagnosis, patients were a median of 73.9 years. AHA was idiopathic in 51.9%; malignancy or autoimmune diseases were associated with 11.8% and 11.6% of cases. Fifty-seven per cent of the non-pregnancy-related cases were male. Four hundred and seventy-four bleeding episodes were reported at presentation, and hemostatic therapy initiated in 70.5% of patients. Delayed diagnosis significantly impacted treatment initiation in 33.5%. Four hundred and seventy-seven patients underwent immunosuppression, and 72.6% achieved complete remission. Conclusions: Representing the largest collection of consecutive AHA cases to date, EACH2 facilitates the analysis of a variety of open questions in AHA.
| year | journal | country | edition | language |
|---|---|---|---|---|
| 2012-04-01 | Journal of Thrombosis and Haemostasis |