A group of hereditarygenetic disorders that impair the body's ability to control blood clotting or coagulation . The effects of this sex-linked, X chromosome disorder are manifested almost entirely in males Females are almost exclusively carriers of the disorder, and may have inherited it from either their mother or father.
Causes Haemophilia A is an X-linked genetic disorder involving a lack of functional clotting Factor VIII and represents 90% of hemophilia cases. Haemophilia B is an X-linked genetic disorder involving a lack of functional clotting Factor IX It is more severe but less common than Hemophilia A. Haemophilia C is an autosomal recessive genetic disorder involving a lack of functional clotting Factor XI.
HEMOPHILIA a Mild : 6 – 50 % of normal factor viii level Moderate : 1 -5 % Severe : < 1% ( joint synovitis , hemophilic arthropathies , IM bleeding, hemarrhagic cyst)
HEMOPHILIA b Genetic bckgrnd , factor level, clinical symptoms are same as HEM A Distinction – 1940 specific factor concentrate
management Severity , type and site of hemorrhage Commercially prepared factor viii & ix complex conc ,desmopressin acetate , cryoppt, ffp…. Chance of viral transmission (dry heat treated concentrate) High purity factor viii products (monoclonal antibody purification technique) - improved viral safety DDAVP (desmopressin acetate) provide transient increase in coag factor. Absence of viral risk and lower cost
ddavp Dose : .3 micro gm/ kg iv or sc prior to tx Result in 2-5 fold increase in factor viii , vwf antigen For children : intranasal spray Stimulate endogenous release f viii&vwf Prolongd use exhaust f viii& dimnishdhemostatcactvty..antifibrinolytcs as adjuncts to ddavp