HEMOPHILIA A
- Factor VIII deficiency, X-linked recessive
- Increased bleeding risk (after minor trauma or surgery)
- High PTT corrected on mixing study, normal PT, normal bleeding time #MedRxed#USMLE#medschool#meded#Medicine
HEMOPHILIA A
- Treatment: Desmopressin (for mild cases), Factor VIII Replacement (for severe cases).
- Patient may develop inhibitor or antibodies to Factor VIII and requires treatment modification..... #MedRXeD#USMLE#ABIM#primarycare#Meded
HEMOPHILIA A w/ Factor VIII Inhibitors
- If inhibitor titer < 5 Bu/ml: Increase dose of FVIII
- If titer > 5: Activated prothrombin complex concentrate, Activated recombinant FVII, Porcine Factor VIII
WE NOW HAVE ONE MORE FDA APPROVED DRUG... #MedRxeD#ABIM#Hematology#Meded
New Drug: EMICIZUMAB
- FDA approved for Hem A w/ factor VIII inhibitors
- bridges activated Factor IX & X
- reduces frequency of bleeding episodes (once weekly inj)
- Warning: thrombotic microangiopathy & thromboembolism on rescue Rx w/ aPCC #MedRXeD#Hematology#Updates#ABIM
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