All are true about haemophilic patient, except:
|B||Raised - PT|
|C||Raised – CT|
a. Inherited deficiencies of all of the coagulation factors are seen, but the three most frequent are factor VIII deficiency (hemophilia A and von Willebrand's diseasE., factor IX deficiency (hemophilia B or Christmas diseasE., and factor XI deficiency. Both hemophilia A and hemophilia B are inherited as sex-linked recessive disorders
b. The clinical severity of both hemophilia A and hemophilia B depends on the measurable level of factor VIII or factor IX in the patient's plasma. Severe disease has factor levels less than 1% of normal, moderately severe disease has levels of 1 to 5%, and mild disease has levels of 5 to 30%.
c. Patients with severe hemophilia have severe spontaneous bleeds, frequently into joints, leading to crippling arthropathies, but these patients also develop intramuscular hematomas, retroperitoneal hematomas, and gastrointestinal and genitourinary bleeding.
d. Mild hemophiliacs do not bleed spontaneously. They are likely to have mild bleeding after major trauma or surgery.
e. Because platelet function is normal and the initial response to an injury is platelet activation and formation of a platelet plug, patients with hemophilia may not bleed immediately after an injury or minor surgery but will begin to bleed several hours later.
f. Treatment of patients with hemophilia A or B is with factor VIII or factor IX concentrate, respectively. Recombinant factor VIII is strongly recommended for patients who have not been treated previously and is generally recommended for patients who are both HIV-and hepatitis C virus (HCV)-seronegative.
g. For factor IX replacement the preferred products are recombinant or high purity factor IX because of the risk of thrombosis with the intermediate purity factor IX (prothrombin complex) concentrates. These concentrates contain varying amounts of factors II, VII, and X and are reported to induce thrombosis when used in high doses.
h. Another option for patients with mild to moderate hemophilia A with minor bleeds is administration of 1-deamino-D-argininevasopressin (DDAVP, desmopressin), which induces the release of vWF from endothelial cells, raising the levels of vWF and associated factor VIII.