Exam 4 Flashcards
What are the 3 different types of bleeding
local or general
mucocutaneous or anatomic
acquired or congenital
Define localized vs generalized bleeding
local-bleeding from a single location
general-bleeding from multiple sites
Define mucocutaneous vs anatomic bleeding
muco-skin or body orifices
ana-bleeding in soft tissue, muscle, joint, deep tissue
What is hematemesis
blood in vomit
Define acquired vs Congenital
acquired-physical trauma induced, not p 2
congenital-acquired at birth
What are the 3 most most common congenital deficiencies in order
VWD
Factor VIII hemophilia A
Factor IX hemophilia B
What does systemic shock lead to
reduction of ADAMTS13
What is the massive transfusion component ratio
6:6:1
6 RBC
6 plasma
1 platelet
What is the first coag facor to decrease activity in liver disease
factor VII
What factor is the best early marker of liver disease
What lab test
prolonged PT- factor VII issue
What factors can be used to differentiate liver disease from vitamin K deficiency
factor V and VII- if decreased means liver disease
if the only VII, means vitamin K
When is there a change in fibrinogen
increases in early or mild liver disase
What factors are unaffected or elevated in liver disease
VWF, F VIII, XIII
How is renal disease related to bleeding deficiencies
fibrinogen is deposited into renal microvasculature, if the kidney fails there is reduced glomerular function
What coag factors are lower in newborns
II, VII, IX, X proteins C, S, Z
How does a vitamin K deficiency or coumadin treatment lead to bleeding
coumadin disrupts enzymes involved in vitamin K coag factors
vitamin K needed for vitamin K dependent factors to create a clot
How do PIVKA factors affect coagulation factors
they inactivate factors II, VII, IX and X and proteins C, S, Z
What lab tests indicate a vitamin K deficiency
prolonged PT
prolonged PTT
What is a common diagnostic of acquired hemophilia
the presence of autoanti fator VIII
What testing is done to find hemophilia
PT, TT, PTT
in mixing studies- the presence of inhibitor
What is the most prevalent congenital mucocutaneous bleeding disorder
VWD
What are the 2 types of VwD
type 1 quantitative
type 2 qualitative
What causes impaired primary hemostasis
decreased plt adhesion to vessel walls
What is the function of VWF
mediates plt adhesion to subendothelial collagen in areas of high flow rate and shear force
Explain the process started by VWF
VWF binds to exposed collagen
plts adhere to GPIb IX/V site to the VWF carpet
plts activated and express GPIIb/ IIIa
allows plt aggregation
severe quantitative VWF deficiency leads to ___ factor deficiency
VIII
Which VWD is most common
Type 1 quantitative
How to ristocetin affect VWF
infolds it and reduces neg charges,
Define hemophilia
congenital single factor deficiency marked by anatomic soft tissue bleeding
What gender does hemophilia mostly occur in
Males
What factor causes hemophilia A
factor VIII deficiency
How does factor VIII affect the coagulation pathway
slows the pathway i its production of thrombin
What chromosome carries the gene of hemophilia A
X chromosome
Males affect all daughters and all sons are carriers
What are the clinical manifestations of hemophilia A
muscle and joint hemorrhages, hematomas, wound oozing and bleeding into CNS
PT, FBG, and TT normal
prolonged PTT
Hemophilia A
What other name does hemophilia B have
christmas disease Factor IX deficiency
What causes hemophilia B
deficiency of factor IX, vitamin K factor dependent.
How does hemophilia B cause bleeding, what type of bleeding
soft tissue anatomic bleeding, reduces thrombin production
PT, TT, FBG normal
PTT prolonged
Factor IX assay
hemophilia B
What other name does hemophilia C have
rosenthal syndrome
factor XI deficiency
PT normal
PTT prolonged
hemophilia C
What populations are more prone to hemophilia C
Ashkenazi Jews
How is
hemophilia A
hemophilia B
hemophilia C treated
A:
B: purified factor IX concentrates
C: plasma infusions
How is
hemophilia A
hemophilia B
hemophilia C treated
A: raise factor VIII
B: purified factor IX concentrates
C: plasma infusions
What does the Russel viper venom time test do
activates coag mechanism at factor X level
can find factor VII deficiency
What test is done to find factor XIII deficiencies
screening assay, suspended in 5 molar urea solution
Define thrombosis
inappropriate formation of a platelet or fibrin clot
What are the 2 main consequences of thrombotic obstructions
ischemia- loss of bleed
necrosis
Define thrombophilia
hypercoagulability, predisposition to thrombosis
Define emboli
fragments of thrombi that move through circulation and get lodged onto veins
What is the most frequent cause of MIs and strokes
arterial thrombosis
2 main examples of venous thrombosis
Pulmonary embolisms and deep vein thrombosis
2 main examples of arterial thrombosis
Myocardial infarction
stroke
What is the most common inherited thrombosis risk factor
factor V Leiden gene mutation
What is the second most common inherited thrombosis risk factor
prothrombin G2021A gene mutation
What is APC resistance
a risk factor: factor V leiden mutation causes factor V to become resistant to APC
What is prothrombin G20210A
a mutation in prothrombin where prothrombin activity becomes elevated
What are antiphospholipid antibodies
APLAs immunoglobulins that bind protein phospholipid complexes
they increase the risk of thrombosis
non specific inhibitor
What are the 3 main types of APLAs in order of importance
lupus anticoagulants LACs
anticardiolipin abs
anti beta GPI abs
What are the signs of APLAs
recurrent fetal loss
thrombocytopenia unexplained thrombosis
What are mixing studies for
to differentiate an LAC from a factor deficiency
PTT -> TT ->repeated then PTT is long, if PTT remains uncorrected
LAC
factor deficiency
factor inhibitor
LAC
PTT long, TT normal,
PTT mix is corrected
next PTT corrects-factor deficiency
next PTT doesn’t correct-factor inhibitor or LAC
What are the 2 most common tests required for an LAC profile
DRVVT- factor X
Silica PTT-factor XII
What result from DRVVT indicates the presence of an LAC
if shortened
>1.2 LAC present
<1.2 must do silica based low phospholipid
Describe APC resistance and why it is important
APC has Arg that resists APC hydrolysis
resistant factor Va remains active and causes clot formation
presence of APC is 3X more thrombosis risk
What is the FVL assay
Factor V Leiden mutation assay to confirm APC resistance
How does G20210A affect hemostasis
elevates plasma prothrombin levels, gene mutation
How does Antithrombin affect hemostasis
SERPIN neutralizes thrombin slows down clot formation
How does PC or PS affect hemostasis
causes recurrent venous thrombosis
increased lipoprotein A mean ___ thrombosis
increased
How does CRP help predict thrombosis
if high, increased risk of MI or stroke
How does fibrinogen affect the changes of thrombosis
if high, increased thrombotic risk
Increased homocysteine levels ___ risk of thrombosis
increase
What are the cardinal symptoms of DVT
edema, erythema, pain, sensation of heat
distended neck veins, tachycardia, shortness of breath
DVT
What tests can predict risk of DVT
D-dimer- if normal rules it out
if high- rules it in
What is DIC
disseminated intravascular coagulation bleeding and clotting at the same time
What occurs to lab tests in DIC
plt count
coag factors
PT/PTT/TT
FSP
D-dimer
plt count- decreased
coag factors- decreased
PT/PTT/TT- prolonged
FSP-increased
D-dimer-increased
What cells are in smears in pts with DIC
schistocytes
What is HIT
Heparin induced thrombocytopenia
immune response to UFH and LMWHabs against platelet factor 4
How does HIT affect hemostasis
reduces plt count,
Venous or arterial thrombosis
lipoprotein a
CRP
homocysteine
high fibrinogen
high VIII
DVT
PE
DVT
PE
venous
everything else arterial
