Inhibitors in Hemophilia A and B

Published on 04/03/2015 by admin

Filed under Hematology, Oncology and Palliative Medicine

Last modified 04/03/2015

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Chapter 61 Inhibitors in Hemophilia A and B

Guglielmo Mariani, Barbara A. Konkle, Craig M. Kessler

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Figure 61-1 BETHESDA ASSAY FOR FACTOR VIII INHIBITOR QUANTITATION.

Relationship between corrected residual factor VIII activity and Bethesda titer is shown. If the result is less than 25%, serial dilutions of the patient’s plasma are tested until the result is between 25% and 75%. The result is multiplied by the dilution to assign the Bethesda titer. One Bethesda unit (BU) is defined by a corrected residual factor VIII of 50% in the assay (dotted line).

(From Konkle BA: Clinical approach to the bleeding patient. In Colman RW, Marder VJ, Clowes AW, et al, editors: Hemostasis and thrombosis, Philadelphia, 2006, Lippincott, Williams & Wilkins, p 1147.)

Treatment of Bleeding in a Patient With FVIII Deficiency and an Inhibitor

Low Titer, Low Responder

Mild Bleeding

• Local and conservative measures, such as rest, ice, compression, and elevation

• If the patient is known to respond to DDAVP (i.e., mild hemophilia A), 0.3 µg/kg IV or 300 µg intranasal (150 µg per nostril; 150 µg for patients <50 kg) for minor bleeding or treatment before minor surgery

• Oral antifibrinolytic therapy (ε-aminocaproic acid or tranexamic acid) for mucosal bleeding

• FVIII dosing to raise the level to 50%

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