11/13 - Hematopoetic and Lymphoid System Flashcards
what are examples of derangement in clotting mechanisms
- hemophilia
- disseminated intravascular coagulation
- excessive fibrinolysis
Inherited or acquired deficiencies of virtually every coagulation factor have been reported as causes of ___
bleeding diatheses
Bleeding due to coagulation factor deficiencies manifest as what
◦ Large posttraumatic ecchymoses
◦ Hematomas
◦ Prolonged bleeding from laceration
◦ After a surgical procedures
bleeding often occurs where in hemorrhagic diatheses
Bleeding often occurs into the GI and urinary
tract and into weight bearing joints
in terms of hemorrhagic diatheses, so hereditary deficiencies affect a SINGLE or MULTIPLE clotting factor
SINGLE
what are the inherited deficiencies that cause hemorrhagic diatheses
- Hemophilia A - Factor VIII
- Hemophilia B - Factor IX
- Von Willebrand factor (both coag and platelet function affected)
in terms of hemorrhagic diatheses, do acquired deficiencies involve SINGLE or MULTIPLE coagulation factors
MULTIPLE
what are acquired deficiencies that can cause hemorrhagic diatheses
◦ Vit K deficiency impairs the synthesis of factors II, VII, IX, X and protein C
◦ Liver disease affects many factors synthesized in liver
hemophilia A also called what
factor VIII deficiency
what is the most common hereditary disease
associated with life-threatening bleeding, is
caused by mutations in factor VIII, an
essential cofactor for factor IX in the
coagulation cascade
hemophilia A
what is trait of hemophilia A? who does it affect?
Inherited as an X linked recessive trait,
hence mainly affects males and homozygous
females
T/F: in 30% of cases of hemophilia A, it is caused by NEW mutation and there is no family history
TRUE
in hemophilia A, patients have [prolonged/normal] PTT and [prolonged/normal] PT
prolonged; normal
Factor VIII deficiency clinical features
what is called the Royal Disease since it appeared in one of Queen Victoria’s sons and was propagated in her descendants
VIII deficiency/hemophilia A