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DR Medhat MRCP

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Acquired Factor VIII Deficiency<br />

- It results from the development of inhibitors against factor VIII coagulation factor.<br />

- It occurs mainly in elderly.<br />

• Association:<br />

Malignancy.<br />

Psoriasis.<br />

Pemphigus.<br />

Drugs : cephalosporins , penicillins<br />

• Diagnosis:<br />

Bleeding tendencies.<br />

APTT: is prolonged (intrinsic pathway).<br />

APTT doesn’t correct/will only correct slightly with the adding of normal<br />

plasma.<br />

Bethesda titre can quantify the inhibitor.<br />

• Prognosis : There is a 20% mortality rate from acquired factor VIII deficiency.<br />

• Treatment:<br />

Replacement of factor VIII is usually ineffective as the inhibitor has rapid<br />

activity.<br />

Recombinant activated factor VII or Factor Eight Bypassing Agent (FEIBA) :<br />

- Used in ttt of bleeding.<br />

- FEIBA is a pooled donor product and is pro-thrombotic, causing<br />

myocardial infarctions and DIC in a subset of patients.<br />

rFVIIa :<br />

- It has been successfully used in patients with acquired hemophilia.<br />

- It binds to the surface of activated platelets, where it supports<br />

thrombin generation and bypasses the need for FVIII.<br />

Definitive removal of the auto-antibody:<br />

- Immunosuppression is successful in at least half of patients, but carries<br />

significant morbidity and mortality in the elderly.<br />

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