Secondary (Clotting Factors) Hemostatic Disorders Flashcards
What are common symptoms of secondary hemostatic (coagulation factor) disorders?
- hemarthrosis
- traumatic ecchymosis/hematoma
- prolonged bleeding from laceration (ie. surgery)
- GI/urinary tract bleeding
- petechiae typically not seen
What is the most common inherited coagulation disorder?
von Willebrand disease
How does vitamin K related to hemostasis?
Vitamin K is required for production of factors II, VII, IX, X and proteins C and S
How does liver failure relate to hemostasis?
Many of the coagulation factors are made in the liver and may be deficient in severe liver failure
-liver is also responsible for activating vitamin K, meaning no vitamin K-dependent coagulation factors
How is the effect of liver failure on coagulation followed?
PT, shows changes earlier from liver failure than other measures
What is hemophilia A?
- deficiency in factor VIII (hemophilia “A-ight/8”)
- X-linked recessive
What lab findings would be expected in hemophilia A?
- elevated PTT (FVIII is in the intrinsic pathway)
- normal PT
- normal platelets
- normal bleeding time
What is the classical presentation of hemophilia A?
-male
*symptoms vary with severity of disease*
-hemarthrosis (very indicative of hemophilia)
-muslce hemorrhages (very indicative of hemophilia)
-petechiae absent
- traumatic ecchymosis/hematoma
- prolonged bleeding from laceration (ie. surgery)
- GI/urinary tract bleeding
What is the treatment for hemophilia A?
recombinant FVIII
What test confirms hemophilia A?
Performance of PTT with patients plasma and with plasma lacking factor VIII identifies if it is factor VIII and not a different intrinsic pathway factor that is deficient.
PTT does not correct in hemophilia A,
What is hemophilia B?
-hemostatic disorder caused by a deficiency in factor IX
-X-linked recessive
What lab findings would be expected in hemophilia B?
- elevated PTT (FIX is in the intrinsic pathway)
- normal PT
- normal platelets
- normal bleeding time
- indisthinguishable from hemophilia A without assay of coagulation factor levels
What is the classical presentation of hemophilia B?
- male
- clinically indistinguishable from hemophilia A
*symptoms vary with severity of disease*
- hemarthrosis (very indicative of hemophilia)
- muslce hemorrhages (very indicative of hemophilia)
- petechiae absent
- traumatic ecchymosis/hematoma
- prolonged bleeding from laceration (ie. surgery)
- GI/urinary tract bleeding
What is the treatment for hemophilia B?
recombinant FIX
What is acquired hemophilia?
- coagulation disorder caused autoimmune Abs against coagulation factors
- FVIII is the most common
- they mimic deficiency in cooresponding coagulation factor