Acquired Hemophilia. Sandeep Kumar Rajan, MD UNMC, Omaha Nebraska. Hemophilia detected later in life. Mild congenital hemophilia in patients with minimal hemostatic stressors. Mild congenital hemophilia with balancing thrombophilia.
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Sandeep Kumar Rajan, MD
UNMC, Omaha Nebraska
What is the diagnosis:
Green D, Lechner K. A survey of 215 non-hemophilic patients with inhibitors to Factor VIII. ThrombHaemost. 1981 Jun 30;45(3):200-3.
AN ANTIBODY THAT NEUTRALIZES THE FUNCTION OR REMOVES A CLOTTING FACTOR FROM CIRCULATION
ALL AUTOANTIBODIES ARE UNCOMMON
Special associations – more
Diagnosis of Clotting Factor InhibitorsMIXING STUDY
FACTOR LEVEL 0% aPTT 80 sec
FACTOR LEVEL 100%
aPTT 28 sec
NOTE: ONLY 30-40% FACTOR
REQUIRED FOR NORMAL aPTT
50% PATIENT : 50% NORMAL
(common doses 90-200 g/kg q 2-6 hrs-not studied)
ERADICATION OF THE INHIBITOR
Efficacy Results at End of Treatment With rFVIIa
92% good/partial response rate with salvage therapy
100% excellent/good response rate with first-line therapy
Hay CRM, et al. Thromb Haemost. 1997;79:1463-1467.
Mayer SA et al. N Engl J Med. 2005;352:777-785.