title: Prognostic factors for remission of and survival in acquired hemophilia A (AHA): results from the GTH-AH 01/2010 study.
abstract: Acquired hemophilia A (AHA) is caused by autoantibodies against factor VIII (FVIII). Immunosuppressive treatment (IST) results in remission of disease in 60% to 80% of patients over a period of days to months. IST is associated with frequent adverse events, including infections as a leading cause of death. Predictors of time to remission could help guide IST intensity but have not been established. We analyzed prognostic factors in 102 prospectively enrolled patients treated with a uniform IST protocol. Partial remission (PR; defined as no active bleeding, FVIII restored >50 IU/dL, hemostatic treatment stopped >24 hours) was achieved by 83% of patients after a median of 31 days (range 7-362). Patients with baseline FVIII <1 IU/dL achieved PR less often and later (77%, 43 days) than patients with ≥1 IU/dL (89%, 24 days). After adjustment for other baseline characteristics, low FVIII remained associated with a lower rate of PR (hazard ratio 0.52, 95% confidence interval 0.33-0.81, P < .01). In contrast, PR achieved on steroids alone within ≤21 days was more common in patients with FVIII ≥1 IU/dL and inhibitor concentration <20 BU/mL (odds ratio 11.2, P < .0001). Low FVIII was also associated with a lower rate of complete remission and decreased survival. In conclusion, presenting FVIII and inhibitor concentration are potentially useful to tailor IST in AHA.
authors.affiliation: Hematology, Hemostasis, Oncology, and Stem Cell Transplantation, Hannover Medical School, Hannover, Germany;
authors.forename: Andreas
authors.initials: A
authors.lastname: Tiede
authors.affiliation: Internal Medicine, Vivantes Clinic Friedrichshain, Berlin, Germany;
authors.forename: Robert
authors.initials: R
authors.lastname: Klamroth
authors.affiliation: Hemostasis, Hemotherapy, and Transfusion Medicine, Heinrich Heine University Center, Düsseldorf, Germany;
authors.forename: Rüdiger E
authors.initials: RE
authors.lastname: Scharf
authors.affiliation: Hematology and Oncology, University Medical Center Schleswig-Holstein, Campus Kiel, Kiel, Germany; Hematology, Hemostasis, Oncology, and Stem Cell Transplantation, Evangelisches Diakonie-Krankenhaus Bremen, Bremen, Germany;
authors.forename: Ralf U
authors.initials: RU
authors.lastname: Trappe
authors.affiliation: Hematology and Oncology, University Hospital Eppendorf, Hamburg, Germany;
authors.forename: Katharina
authors.initials: K
authors.lastname: Holstein
authors.affiliation: Hemophilia Care Center, SRH Kurpfalzkrankenhaus Heidelberg, Heidelberg, Germany;
authors.forename: Angela
authors.initials: A
authors.lastname: Huth-Kühne
authors.affiliation: Internal Medicine, Vivantes Clinic Friedrichshain, Berlin, Germany;
authors.forename: Saskia
authors.initials: S
authors.lastname: Gottstein
authors.affiliation: Institute for Clinical Chemistry and Laboratory Medicine, Freiburg University Hospital, Freiburg, Germany;
authors.forename: Ulrich
authors.initials: U
authors.lastname: Geisen
authors.affiliation: Clinical Hemostaseology and Transfusion Medicine, Saarland University Hospital, Homburg/Saar, Germany;
authors.forename: Joachim
authors.initials: J
authors.lastname: Schenk
authors.affiliation: Center of Coagulation Disorders, Leipzig, Germany;
authors.forename: Ute
authors.initials: U
authors.lastname: Scholz
authors.affiliation: Hematology and Oncology, Jena University Hospital, Jena, Germany;
authors.forename: Kristina
authors.initials: K
authors.lastname: Schilling
authors.affiliation: Hematology, Graz Medical University, Graz, Austria;
authors.forename: Peter
authors.initials: P
authors.lastname: Neumeister
authors.affiliation: Hematology and Oncology, Johann Wolfgang Goethe University Hospital, Frankfurt/Main, Germany;
authors.forename: Wolfgang
authors.initials: W
authors.lastname: Miesbach
authors.affiliation: Clinical Chemistry, University Medical Center Schleswig-Holstein, Campus Lübeck, Lübeck, Germany;
authors.forename: Daniela
authors.initials: D
authors.lastname: Manner
authors.affiliation: Hematology, Hemostasis, and Oncology, Paracelsus Medical University, Salzburg, Austria;
authors.forename: Richard
authors.initials: R
authors.lastname: Greil
authors.affiliation: Hematology and Oncology, Mainz University Hospital, Mainz, Germany;
authors.forename: Charis
authors.initials: C
authors.lastname: von Auer
authors.affiliation: Internal Medicine, German Diagnostic Clinic, Wiesbaden, Germany;
authors.forename: Manuela
authors.initials: M
authors.lastname: Krause
authors.affiliation: Anesthesiology, Evangelical Hospital, Bielefeld, Germany;
authors.forename: Klaus
authors.initials: K
authors.lastname: Leimkühler
authors.affiliation: Transfusion Medicine, Charité University Hospital, Berlin, Germany;
authors.forename: Ulrich
authors.initials: U
authors.lastname: Kalus
authors.affiliation: Hematology, Hemostasis, Oncology, and Stem Cell Transplantation, Hannover Medical School, Hannover, Germany;
authors.forename: Jan-Malte
authors.initials: JM
authors.lastname: Blumtritt
authors.affiliation: Hematology, Hemostasis, Oncology, and Stem Cell Transplantation, Hannover Medical School, Hannover, Germany;
authors.forename: Sonja
authors.initials: S
authors.lastname: Werwitzke
authors.affiliation: Biometry, Hannover Medical School, Hannover, Germany; and.
authors.forename: Eva
authors.initials: E
authors.lastname: Budde
authors.affiliation: Biometry, Hannover Medical School, Hannover, Germany; and.
authors.forename: Armin
authors.initials: A
authors.lastname: Koch
authors.affiliation: Hematology and Hemostasis, Vienna Medical University, Vienna, Austria.
authors.forename: Paul
authors.initials: P
authors.lastname: Knöbl
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001323:Autoantibodies / Q000009:adverse effects; D005169:Factor VIII / Q000032:analysis / Q000276:immunology; D005260:Female; D006467:Hemophilia A / Q000175:diagnosis / Q000401:mortality / Q000628:therapy; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012074:Remission Induction; D016019:Survival Analysis; D016896:Treatment Outcome
chemical_list: D001323:Autoantibodies; D007166:Immunosuppressive Agents; C427184:recombinant factor VIII SQ; D005169:Factor VIII