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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