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The two most common types are hemophilia A and hemophilia B. Over 80 per cent of patients have hemophilia A (classical hemophilia), which is characterized by a deficiency of coagulation factor VIII. Hemophilia B (called also Christmas disease) affects about 15 per cent of hemophiliacs and is characterized by a deficiency of coagulation factor IX. Other coagulation factor deficiencies are less common, with patients suffering either milder bleeding or thrombotic episodes.
Hemophilia A is characterized by a factor VIII level of from 0 to 30 per cent of normal. The partial thromboplastin time (PTT) is usually prolonged. The platelet count, bleeding time, and prothrombin time (PT) are normal. In hemophilia B there is a low factor IX level and the prothrombin time is usually prolonged.
All persons with hemophilia should be immunized with hepatitis B vaccine. Suspected bleeding into the central nervous system must be promptly treated; when this happens, consultation with or transfer to a Hemophilia Treatment Center is mandatory. Early treatment of hemarthroses is essential for maintenance of joint health; infusions administered within 4 to 6 hours of onset of symptoms are sufficient to stop bleeding and restore joint function. Medically supervised home infusion therapy has become an integral part of the comprehensive care of patients with bleeding disorders and has facilitated the treatment of bleeding episodes outside the hospital setting.
Excellent sources of information for both professionals and nonprofessionals are the National Hemophilia Foundation, 110 Greene St., Suite 303, New York, NY 10012 (telephone 212-219-8180) and the Hemophilia and AIDS/HIV Network for the Dissemination of Information (“HANDI”) at the same address (telephone 800-42-HANDI).
Management Factor IX concentrates
hemophilia BChristmas disease, factor IX hemophilia Hematology An X-R form of hemophilia caused by a defect in factor IX; it is clinically similar to hemophilia A, and often first manifest by hemarthrosis with joint destruction; these Pts are often infected with blood-borne viruses–eg, HBV, HCV, HIV, parvoviruses; with time, IgG antibodies may develop against factor IX. See Hemophilia. Cf Thalassemia.
Christmas,Stephen, patient with the first case of this disease studied in detail.
he·mo·phil·i·a B(hē'mō-fil'ē-ă) [MIM*306900-various]
Patient discussion about hemophilia B
Q. A christmas wish for each of you! To have all the presents in the world is not my desire for you! May your Christmas gift from me today be a sincere prayer that you will know: Peace that makes you sleep like a baby Joy that makes you laugh like a fool Happiness that lets you forget the size of your problems Hope that makes impossible dreams come true Love that makes you give from your heart Forgiveness when ‘you know not what you do”! Kindness that brings favor wherever you go And health that makes you act the age you choose.
Stay healthy always..