Defects of Plasma Clotting Factors Flashcards
Bleeding is categorized as? (2)
Give examples
Open
- Tounge
- Tonsil
- Gums
- Epistaxis
Closed
- Soft tissue
- Genitourinary
- Gastrointestinal
- Bleeding in muscles, bones, joints, skull
Define the Factor VIII molecule? (5)
- Largest protein in coagulation complex
- Not synth exclusively by liver
- X-chromosome controlled
- complexes w/ vWF for transport
- Labile in stored plasma
Ddescribe the Hemophilia Disorders?
Any disorders in where a particular clotting factor is decreased.
What are the two classic sex-linked recessive hemophilia disorders?
Population frequency percentage?
What factor is deficient?
-
Hemo A (85%)
- Factor VIII
- 20 - 30% no family history
-
Hemo B (10 - 14%)
- Factor IX
What is tghe minimum percentage of factors required to achieve hemostasis?
30%
Bleeding manifestations are related to the level of clotting factor activity? (percentages)
- Severe <1%
- Moderate 1 - 5%
- Mild 6 - 24%
What is Hemoarthrosis?
Bloody effusion inside joint
In Hemophilia A and B what lab results are abnormal and normal?
aPTT imcreased
Normal PT and BT
What is the dilution for pagtient plasma for an APTT test?
1:10
Describe early treatment and current treatment for hemophilia A? What disease did they contract?
Early: whole blood transfusions, cryoprecipitate contains factors VIII and vWF.
- 80-90% contracted HIV
Current is with recombinant products: Factors VIII and IX available
What is the treatment for Hemophilia B?
- Factor IX concentrates
- Prothrombin complex (X, IX, VII, II)
Clotting factor disorders: with bleeding manifestations
- Defeciencies of which factors are rare?
- How is PT and APTT affected?
- What are the genetics?
- What is the treatment for patients deficient in II, VII, and X?
- II, V, VII, X (Common Pathway minus factor I and Extrisic pathway)
- Prolonged
- Autosomal recessive
- Prothombin complex concentrates
Clotting factor disorders: with bleeding manifestations
Factor XIII - Transglutaminase
- How does this affect PT and APTT?
- Increased chance of what during pregnancy
- Without Factor XIII clot ___ ____
- What affects are seen during inury?
- Normal
- Miscarriage and spontaneous abrotion
- doesn’t stablize
- prolonged bleeding w/ minor injuries, keloid scarring
Factor XIII deficiency can be assiciated with which diseases/disorders? (4)
CLUD
- Chron’s
- Leukemias
- Ulcerative colitis
- DIC (disseminated intravascular coagulation)
How is Factor XIII deficiency tested for?
5M Urea Test
- measures stability of firmness of clot after 24hrs in 5M urea
