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... ecombinant DNA technology and the discovery of the genes that control production of factor VIII have led to the development of recombinant factor concentrates that do not rely on plasma at all. What is the Cause of Hemophilia? Hemophilia occurs when one of the factors needed for blood to clot is missing or the amount present is too low for normal clotting to occur. Factor VIII is missing or too low in about 90 % of hemophilia cases.
Factor IX is deficient in about 9 % of cases. Factor XI, X, VII or V is missing or too low in the remaining 1 % of hemophilia cases. The process of blood clotting starts the moment damage to the blood vessel wall occurs. Platelets begin collecting at the damaged site.
As more platelets collect, a plug forms in the hole to stop bleeding. Although the platelet plug can hold for a while, without a 'glue' to permanently hold the platelets together, the plug breaks apart and bleeding begins again. The 'glue' that holds the plug together is called fibrin. Fibrin is the end product of two blood clotting pathways. Each pathway proceeds in a cascade or stepwise fashion with the product in one step acting as the trigger for the next step.
These pathways are diagram below. The first pathway (extrinsic system) is triggered when damage causes a substance called tissue thromboplastin to be released from the tissue. The second pathway (intrinsic system) is triggered when the blood comes into contact with the blood vessel wall. The steps that lead to the formation of fibrin requires factors VIII and IX. The body produces the different clotting factors based on the instructions from a gene. In hemophilia, the gene that causes factor to be produced is defective.
In most cases of hemophilia, the defective gene is passed from parent to child. In cases where there is no family history of the disorder, hemophilia occurs due to a change or mutation of the gene. In such cases, the mutation may only be discovered after the birth of a son with hemophilia. How is Hemophilia Diagnosed? The diagnosis of hemophilia is made through a series of tests on a sample of the patient's blood.
The pattern of positive and negative results from different tests will diagnose the presence of hemophilia as well as the type. The tests listed below are used to diagnose hemophilia. Platelet count: This test counts the number of blood platelets. This test is normal for patients with hemophilia. Activated partial thromboplastin time (APTT): This test diagnoses problems with Factor VIII and Factor IX. Almost 100 % of patients with severe and moderate Factor VIII deficiency can be diagnosed with an APTT.
Diagnosis of mild cases varies depending on the type of materials used in testing the blood sample. Most carriers cannot be diagnosed with an APTT. The test measures the length of time that it takes for a blood clot to form. The activated partial thromboplastin time is longer than normal for patients with Factor VIII Deficiency (Hemophilia A) and Factor IX Deficiency (Hemophilia B).
Factor assay: This is the most exact test to diagnose the type of hemophilia. A factor assay can distinguish between a Factor VIII deficiency and a Factor IX deficiency. Adding normal serum to the patient's serum will correct the abnormal APTT test result for Factor IX deficiency. Adding normal plasma to the patient's serum will correct the abnormal APTT test result for Factor VIII deficiency. How Blood Clots To understand inhibitors, it helps to know how blood normally clots.
When blood starts to flow out of a damaged blood vessel, the process is called coagulation. Coagulation is the body's way of sealing a leak. The seal, or clot, consists of red and white blood cells and platelets, which cling to threadlike material called fibrin. Twelve clotting factors must work in sequence to produce wrought fibrin to make a strong clot. The coagulation process can be compared to a domino effect (figure 1). Each clotting factor in the blood must activate the next one in the series in order to form a clot.
When there is an insufficient amount of any one of these clothing factors, the process stops permatuerly, interrupting the production of fibrin. In most people with Hemophilia, replacement clotting factor can compensate for the deficient clotting factor, enabling the clotting process to continue. For people with inhibitors, replacement factor may be inactivated befor it ahs a chance to work. Anti hemophilic Factor Anitemophilic factor (human) Method M monoclonal purified monarc-M is a sterile, non pyogenic, dried preparation of anti hemophilic factor (factor VIII, factor VIII: C AHF) in concentrated form with a specific activity rang of 2 to 15 AHF international units / mg of total protein.
When reconstituted with the appropriate volume of diluent, it contains approximately 12. 5 mg / ml Albumin (human), 1. 5 mg / ml polyethylene go; (3350), 0. 055 M histidine and 0. 03 M glycine as stabilizing agents. In the absence of the added Albumin (human), the specific activity is approximately 2, 000 AHF international Units / mg of protein. It also contains, per AHF international Units, not more the 0. 1 ng mouse protein, 18 ng organic solvent (tri-n-butyl phosphate) and 50 ng detergent (octoxynol 9) Monarc-M is prepared by the method M process from pooled human plasma by immuno affinity chromatography utilizing a murine monoclonal antibody to factor VIII: C, followed by an ion exchange chromatography step for further purification. Monarc-M also includes an organic solvent (tri-n-butyl phosphate) and detergent (octoxynol 9) virus inactivation step designed to reduce the risk of transmitting of hepatitis and other viral diseases. However, no procedure has been shown to be totally effective in removing the viral infectivity fron coagulation factor products.
Monarc-M can ONLY be administered intravenously and takes approxmiatly 12 - 14 hours befor the factor begins to work and the clothing factor in the blood is brought up. After 24 - 28 hours after that the factor is wiped out of the blood, befor this time the Factor is leaving the blood it is at this time that ALL of the factor is gone, and the person rooms there blood clothing factor in there blood. Factor Survival Rate Clotting factor normally circulates at constant levels in the blood and only works when an injury occurs. We clotting factor replacmnet is given with factor concentrate, fresh frozen Plasma or cryoperecipitate, the factor only survives for a short time. It's different for every Hemophiliac. Your hematologist can find out by doing a test called a factor Survival or Recovery.
For factor VIII, Half of the dose given will be used up in 4 Hours, in another 8, it will again be reduced by half and so on untill it is all used up. Factor IX, given as plasma or factor IX concentrate, usually last a little longer. Factor survival studies are not usually needed for everyday treatment, but befor major surgery we of ike patients to be tested. Blood samples are draw befor and after the administration of the clotting factor. this helps us decide on the dose of factors necessary for control of bleeding following surgery.
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