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What deficiency in clotting factors causes hemophilia A? |
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Definition
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What clotting factor deficiency causes Hemophilia B? |
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Definition
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How are pts with Hemophilia classified? |
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Definition
- Hemophilia A (factor VIII deficient) - most common
- Hemophilia B (factor IX deficient)
- Both sex linked dxs- found on X chromosome
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Who is most commonly affected by hemophilia? |
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Definition
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What is the normal presenting factors for hemophilia? |
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Definition
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How is Hemophilia A diagnosed? |
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Definition
Ideally the APTT is sensitive to factor deficiency states <30% activity. • Patients with a normal PT but an abnormal APTT typically have deficiencies of factors that are unique to the intrinsic system (factors VIII, IX, XII, and XI). • The specific factor assay for factor VIII or IX will reveal the appropriate defect. • the exception is in hemophilia Bm in which the PT and APTT are prolonged. • ALL patients with factor VIII deficiency should undergo test for Von Willebrand’s disease. |
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what is the treatment for Hemophilia A? |
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Definition
Those with severe or moderate disease are treated with either cryoprecipitate or factor VIII concentrate. Two sources of factor VIII concentrate: Plasm Derived Recombinant factor VIII Factor is qualified in units: 1 unit of Factor VIII is same # of units in 1 ml of pooled PLTS
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Term
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Definition
Rich in factor VIII, fibrinogen, and Von Willibrands factor each bag contains 70-100 units of factor VIII
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Term
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Definition
used to treat mild factor VIII deficiencies transiently increases factor VIII and von Willibrands factor- shortend prolonged bleeding times synthetic analouge of vasopressin
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Indications for use of desmopressin? |
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Definition
- treat mild factor VIII deficiencies
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Side effects of desmopressin? |
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Definition
Facial flushing (most common) HA Increased HR Decreased BP Water retention- hyponatremia increases plasimogen activator
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Recommended treatment for pts with Inhibitors? |
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Definition
Antibodies to factors VIII and factor IX, also known as inhibitors can develop in hemophiliac patients. • Should be suspected when calculated dose does not result in the desired increase in factor level. • Treatment can be approached by different methods –Reduce inhibitor levels with immunosuppresent therapy (cyclophosphamide), gamma-globulin, plasmopheresis, or by tolerance with high dose or frequent administration of factor administered During acute bleeding or Surgery • Patients with low titers <5 BU/ml –Give double usual dose of factor • Patients with high titers >10 BU/ml –Give (PCC) Prothrombin complex concentrate or (APCC) activated prothrombin complex concentrate –Porcine factor VIII Usual dose 50-75 units /Kg every 12 hours. –Alternative is recombinant factor VIIa |
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Term
How does hemophilia Bm differ from Hemophilia B? |
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Definition
PT and aPTT are prolonged in Bm |
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Recommened treatment for Hemophilia B? |
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Definition
• High purity factor IX plasma concentrate is currently the treatment of choice of hemophilia B. • Replaced older products, known as PCC with also contained vitamin K dependant cofactors responsible for thromboembolic complications associated with their use. • Half life of factor IX is 24 hours, with normal hemostasis occuring with plasma levels are about 10-25%. • Each unit of factor IX infused per Kg increases factor IX plasma levels 1% |
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Definition
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von Willibrands DX is classified how? |
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Definition
quantitative qualitative or both
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How is von Willibrands DX treated? |
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Definition
Goal to improve bleeding time Cryoprecipitate Humate-T, Koate-HS, Koate-HP IV DDAVP (for mild vWD)
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