Patern of Acquired Hemophilia in Saudi Arabia

Background: Acquired Hemophilia (AH) is a rare bleeding disorder that develops secondary to an autoantibody against Factor VIII in persons who are not known to have any previous bleeding disorder. Aim: The aimof this retrospective case review is to explore the pattern of confirmed cases of AH that were managed in Saudi Arabia over a 5 years' period.

Patients and Methods: We reviewed the charts of documented cases of AH that were managed in 5 Tertiary Hospitals (4 in Riyadh and 1 in Al Madinah Al Monawarah) from 2010 to 2014. All cases underwent medical history, physical examination, laboratory tests including complete blood count, activated partial thromboplastin time (PTT), mixing studies, factor VIII level, and Factor VIII Inhibitor titer. All patients received standard treatment.

Results: We found 19 cases, but we could obtain data only for 13 cases, 4 males and 9 females, median age was 30 years (range 20-85). Risk factors were pregnancy in 5, Autoimmune disease in 2, Cancer in 1, Clopedogril in 1 and 4 were idiopathic. All cases presented with various degrees of bruises. in addition, 4 cases presented with menorrhagia, 4 cases with soft tissue hematomas, and 2 cases with gross hematuria. All cases had prolonged aPTT, which was only partially corrected with mixing studies, very low factor VIII level (median 1, range <1-12.5%), and high factor VIII inhibitor titer (median 22.4 BU, range 3.5-1932). Treatment included steroids, cyclophosphamide, immunoglobulins and Rituximab. There were 9 remissions (with 2 relapses, which responded to treatment), 2 partial responses, and 2 deaths due to massive blood loss (Hematuria).

Discussion: Our case series indicates only severe AH, because many milder cases are underdiagnosed or misdiagnosed. Although the number of our cases are small, they indicate a different pattern from the published studies, because of younger age group and female predominance.

Conclusion: Our case series indicates that most of our cases are young adults, with female predominance. A comprehensive review of cases from other hospitals is underway