bleeding problem - psych80

[ArchivalUser]

Psych sorry Bro but something is wrong with your Q...you can't infuse porcine factor without stopping first Factor VIII.

[ArchivalUser]

@psych..if its factor def..then mixin wud correct PT/PTT..while if it does'nt correct PT/PTT..then antibody should be suspected..plz correct me if wrong..n cud u post d explanation too..thx!

[ArchivalUser]

(F) Porcine factor VIII

Explanation:

The patient presents with spontaneous hemiarthrosis, which is a classic presentation of patients with severe hemophilia. Depending on the severity of the disease, patients can present with bleeding into the joints, muscles, or gastrointestinal tract. This can occur spontaneously if the factor VIII level is extremely low or after minimal trauma at higher levels of activity. Hemophilia A is seven times more common than is hemophilia B (factor IX deficiency).

The failure of the factor VIII:C level to increase significantly after 24 hours of factor VIII replacement therapy, along with the failure of the PTT to correct on the plasma mixing study, indicate the presence of a factor VIII inhibitor. Antibodies to factor VIII may develop in 15% of patients with hemophilia who have received infusions of factor VIII concentrate. Factor VIII inhibitor may also develop postpartum or could be idiopathic. The treatment of choice is porcine factor VIII. Porcine factors have different antigens compared with recombinant factors. It will not be destroyed by the same anti-factor VIII antibodies that are active against recombinant factor VIII. The Bethesda titer is a way to quantify the amount of factor VIII antibodies present in plasma. The higher the titer, the greater the amount of factor VIII antibody present. When the antibody is present in low titer (5), you can use factor IX concentrates.

Cyclophosphamide and prednisone as a combination is antiquated therapy. Although they will eventually lower the antibody level, they will not work acutely enough to be very useful to this patient who presents with acute bleeding. Plasmapheresis and aggressive factor VIII therapy are also useful treatments. Immunoglobulin therapy is not currently indicated in the treatment of factor VIII inhibitor. Obtaining a factor IX level is not indicated when we already have a low factor VIII level on record. Desmopressin acetate is used most often in patients with mild hemophilia (factor VIII:C >5%) in preparation for minor surgical and dental procedures. Desmopressin is unlikely to be effective when the factor VIII levels are under 5%.

[ArchivalUser]

algeria92, the question asks what is the best step in management NOT next best step .. ofcourse we have to stop to factor VIII because its not useful .. but do you think its HARMFUL for the patient .. i dont think so ..

[ArchivalUser]

@ km2 .. you r right

[ArchivalUser]

good question n lovelt explanation.
plasmapheresis was the option i was searching for.
i thought the antibodies would cross react wid porcine factor VIII too.

[ArchivalUser]

n hey pasyc..
no confusion regarding prednisolone coz we need to treat the case acutely. prednisone will take long time to show effect because by blocking antibody production.

[ArchivalUser]

hohooooo...sorry Psych, but still not convinced by your explanation still think that the best management here ( mild bleeding) is First To stop Factor 8 to prevent an anamnestic response, which would preclude this treatment from being a treatment option in the event of future bleeding episodes.

in case of Severe bleeding episodes
Use human or porcine FVIII until an anamnestic response occurs, after which a bypass agent must be used instead.