Hemarthrosis and soft tissue hematomas are seldom found in these patients.4, 8, 11 Patients with hemophilia usually do not bleed abnormally from small cuts such as razor nicks. After larger injuries, however, bleeding out of proportion to the extent of injury is common. This bleeding may be massive and life threatening or it may persist as a slow, continuous oozing for days, weeks, or months. The onset of excessive bleeding usually is delayed. At the time of surgery or injury, hemostasis appears to be normal. Bleeding of sudden onset and serious proportions may develop several hours or even several days later. Venipuncture, if skillfully performed, is of no danger to the patients with hemophilia because of the elasticity of the venous walls.
Screening tests that show prolonged apt, normal PT, and normal platelet count (except in some cases of von Willebrand disease) indicate a problem in the intrinsic pathway. The next step is to mix (mixing tests) the patient’s .The swelling in the submandibular region in a patient with hemophilia was caused by bleeding after intraoral trauma.
B, The floor of the mouth has been elevated because of the bleeding. (From Hoffbrand AV: Color atlas of clinical hematology, ed 3, St. Louis, 2000, Mosby.) Blood with a sample of pooled plasma and repeat the aPTT. If this test result is normal, then the specific missing factor is identified by specific assays. If the mixing test result is abnormal, tests for inhibitor activity (antibodies to the factor) are performed the lifetime risk of death from intracranial hemorrhage is 2% to 8%.
More than 75% of adults with hemophilia A and 45% of adults with hemophilia B are HIV positive. The anti-HIV protease inhibitors result in prolonged HIV disease survival among this group of patients. With the exception of HIV and HCV infection, life expectancy is related to the severity of hemophilia
, and the mortality rate is four to six times higher for severe disease than for mild to moderate disease. The mortality rate among patients with inhibitors is much greater than among those without inhibitors.2, 8