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
what is hemophilia B also called
Christmas disease (named after person not holiday) and Factor IX deficiency
what disease:
Severe factor IX deficiency produces a
disorder clinically indistinguishable from
factor VIII deficiency
hemophilia B
what is the trait of hemophilia B
inherited X linked recessive
does hemophilia B show variable clinical severity
YES
is PTT in hemophilia B NORMAL or PROLONGED
prolonged
how to tx hemophilia B
infusion of recombinant factor IX
A bleeding disorder caused by the qualitative
or quantitative deficiency of the von
Willebrand factor
Von Willebrand Disease
Von Willebrand factor necessary for proper ___
platelet adhesion to damaged blood vessels
what is a carrier for Factor VIII and protects it from degradation
Von Willebrand factor
affected ppl with von willebrand disease sypmtoms
◦ Excessive bruising
◦ Prolonged bleeding from mucosal surfaces
◦ Prolonged bleeding after minor trauma
what type of vWF is associated with QUANTITATIVE defects
Type 1 and 3
what type of vWF is characerized by QUALITATIVE DEFECTS
Type 2
do ppl with vWF have mild, moderate or severe symptoms
MILD
symptoms of vWD? is this commonly diagnosed?
often underdiagnosed!
◦ Women may have heavy menstrual periods
◦ Epistaxis
◦ Excessive bleeding from wounds
who gets vWD? is this AD or AR?
AD or AR; both men and women can have it in
about equal numbers
what is an acute, subacute, or chronic thrombohemorrhagic disorder characterized by the excessive activation of coagulation and the formation of thrombi in the microvasculature
disseminated intravascular coagulation
what is disseminated intravascular coagulation also called
DIC or consumptive coagulopathy
because of thrombotic diathesis, what happens in DIC
because of thrombotic diathesis, there is consumption of platelets, fibrin, and coagulation factors, and secondarily activation of fibrinolysis
DIC signs and symptoms
◦ Tissue hypoxia and infarction caused by microthrombi
◦ Hemorrhage due to depletion of factors required for hemostasis and activation of fibrinolytic mechanism or both
common triggers of DIC
◦ Sepsis
◦ Major trauma
◦ Certain cancers
◦ Obstetric complications
tx for DIC
anticoagulants
