Contributions
Abstract: PB1807
Type: Publication Only
Background
Acquired haemophilia A (AHA) is a rare autoimmune disease, caused by antibodies (inhibitors) against coagulation FVIII and characterized by spontaneous hemorrhage in patients with no previous history of bleeding. Risk factors for the occurrence of AHA include advanced age and underlying diseases (malignancy, autoimmune disorders, pregnancy, and the postpartum period).
Aims
In this work we showed how the underlying diseases of acquired haemophilia A affect the treatment outcome and prognosis (deaths in patients).
Methods
We analyzed the results of fifteen patients (10 (66,7%) male,5 (33,3%) female), median age 71 (51-81) with AHA from July 2010 until December 2017, treated at the UHC Zagreb. Severe bleeding had 66,7% patients. Almost half of patients (46,7%) had FVIII activity < 5 U/ dL. The median inhibitor titre at diagnosis was 5,8 (2,3–2000) BU/mL. In 7 patients (46,7%) were not identified underlying diseases associated with AHA. In 8 patients (53,7%) were identified underlying diseases; malignancies 3 (oligodendroglioma, B-CLL, IgM monoclonal gamapathy), autoimmune diseases 4 (autoimmune haemolytic anaemia in 2 patients, rheumatoid arthritis, polymyalgia rheumatica), 1 postinfective.
Results
Haemostatic therapy was initiated in 86,7% of patients (100% with severe bleeding) with AHA. First-line therapy consisted of bypassing agents (recombinant FVIIa in 13,3%, activated prothrombin complex concentrates in 73,3%), and all patients were treated with immunosupressive treatment (combination of steroids and cyclophosphamide in 13 patients, steroids alone in 2 patients). Rituximab alone was used in one patient as the second line treatment. Both group with underlying and without underlying disease were similar according to the median age 71, FVIII activity 5 vs 6 U/dL, time to achieve remission was the same 28,2 days. The titre inhibitor was significantly higher in the group with underlying disease (22 vs 3,5 BU/ml), and the rate of major bleeding was higher (86,7 vs 42,6%). At follow up of 31 months (1-71) 11 patients (73,3%) are alive, 4 (26,6%) dead. No death was due to bledding. Three (75%) of deaths was reported in a group of patients with underlying disease (two deaths associated with sepsis, one with progressive malignant disease) with mortality rate of 37,5 vs 14,2% on behalf of group with known cause of AHA. One death was associated with cardiogenic shock in patient without underlying disease of AHA. One patient (7%) had thrombotic complication (myocardial infarction and venous thromboembolism).
Conclusion
Mortality in patients with acquired haemophilia was associated with the underlying disease, cause of AHA (malignant disease), as well as with infective complications of immunosupresive treatment in elderly with comorbidites, according to recent literature data.
Session topic: 34. Bleeding disorders (congenital and acquired)
Keyword(s): Acquired factor VIII inhibitor, Acquired hemophilia
Abstract: PB1807
Type: Publication Only
Background
Acquired haemophilia A (AHA) is a rare autoimmune disease, caused by antibodies (inhibitors) against coagulation FVIII and characterized by spontaneous hemorrhage in patients with no previous history of bleeding. Risk factors for the occurrence of AHA include advanced age and underlying diseases (malignancy, autoimmune disorders, pregnancy, and the postpartum period).
Aims
In this work we showed how the underlying diseases of acquired haemophilia A affect the treatment outcome and prognosis (deaths in patients).
Methods
We analyzed the results of fifteen patients (10 (66,7%) male,5 (33,3%) female), median age 71 (51-81) with AHA from July 2010 until December 2017, treated at the UHC Zagreb. Severe bleeding had 66,7% patients. Almost half of patients (46,7%) had FVIII activity < 5 U/ dL. The median inhibitor titre at diagnosis was 5,8 (2,3–2000) BU/mL. In 7 patients (46,7%) were not identified underlying diseases associated with AHA. In 8 patients (53,7%) were identified underlying diseases; malignancies 3 (oligodendroglioma, B-CLL, IgM monoclonal gamapathy), autoimmune diseases 4 (autoimmune haemolytic anaemia in 2 patients, rheumatoid arthritis, polymyalgia rheumatica), 1 postinfective.
Results
Haemostatic therapy was initiated in 86,7% of patients (100% with severe bleeding) with AHA. First-line therapy consisted of bypassing agents (recombinant FVIIa in 13,3%, activated prothrombin complex concentrates in 73,3%), and all patients were treated with immunosupressive treatment (combination of steroids and cyclophosphamide in 13 patients, steroids alone in 2 patients). Rituximab alone was used in one patient as the second line treatment. Both group with underlying and without underlying disease were similar according to the median age 71, FVIII activity 5 vs 6 U/dL, time to achieve remission was the same 28,2 days. The titre inhibitor was significantly higher in the group with underlying disease (22 vs 3,5 BU/ml), and the rate of major bleeding was higher (86,7 vs 42,6%). At follow up of 31 months (1-71) 11 patients (73,3%) are alive, 4 (26,6%) dead. No death was due to bledding. Three (75%) of deaths was reported in a group of patients with underlying disease (two deaths associated with sepsis, one with progressive malignant disease) with mortality rate of 37,5 vs 14,2% on behalf of group with known cause of AHA. One death was associated with cardiogenic shock in patient without underlying disease of AHA. One patient (7%) had thrombotic complication (myocardial infarction and venous thromboembolism).
Conclusion
Mortality in patients with acquired haemophilia was associated with the underlying disease, cause of AHA (malignant disease), as well as with infective complications of immunosupresive treatment in elderly with comorbidites, according to recent literature data.
Session topic: 34. Bleeding disorders (congenital and acquired)
Keyword(s): Acquired factor VIII inhibitor, Acquired hemophilia