Coagulation Disorders Flashcards
what are the VItamin K dependent factors?
II (prothrombin), VII, IX, X
what are the thrombin sensitive factors
I (fibrinogen), V, VIII, XIII
what is the Regulatory mechanism for maintaining blood flow
fibrinolytic system
when is plasminogen release?
in response to thrombin, venous stasis, exercise, ischemia
what is the extrinsic clotting pathway? Time to fibrin formation after addition of thromboplastin to plasma. Used w/ INR
PT- Prothrombin time
what is INR
International normalized ratio
ratio of PT patient/ PT normal
what cause elevated INR?
Liver disease
Vit K deficiency
Warfarin
what is a normal INR
0.8-1.2
what is aPTT
activated partial thromboplastin time
intrinsic and common pathways
When is aPTT elevated?
Liver dz
heparin
hypercoaguability
what does D-dimer tell you?
that plasma is working
when is a D-dimer positive?
DVT/PE
Disseminated intravascular coagulation (DIC)
what does heparin activate?
antithrombin III
what is the most common congenital bleeding disorder?
Von Willebrand Disease
What do patients w/ Von Willebrand Dz have decreased quantity of?
Von Villebrand factor
which stabilizes Factor VIII
What pathway is affected by VOn Willebrand Dz
intrinsic
what are the three types of Von Willebrand Dz
Mild to Moderate (most common)
Functionally abnormal
Nearly undetectable (most severe)
what is acquired VW dz associated w/?
autoimmune dz
what drugs are associated w/ acquired VW dz?
valproic acid
griseofulvin
hydroxyethyl starch (volume expander)
ciprofloxacin
S/S of VW dz
mucocutaneous bleeding
easy bruising
post-op bleeding
what lab will be abnormal w/ VW dz?
aPTT will be prolonged VWF low (PT and platelets are w/i normal range)
tx for VW dz?
apply topical thrombin (surgery)
GIve exogenous VW factor or Factor VIII
What drug can be given for VW dz?
Desmopressin (DDAVP)
stimulates release of VFW and factor VIII
ADRs of desmopressin
tachycardia
HA
flushing
what to monitor when giving desmopressin
pulse, BP
Factor VIII levels
aPTT
bleeding time
what are antifibrinolytic therapies for VW dz? Often used as an adjunct
aminocaproic acid tranexamic acid (surgical preps, for non coag problems)
what do you need to use caution w/ for aminocaproic acid and tranexamic acid?
Renal dysfunction
ADRs of aminocaproic acid and tranexamic acid?
Diarrhea
N/V
HPOTN
Thrombosis/ DVT
what is used in patients unresponsive to DDAVP?
WVF/Factor VIII concentrates
ADRs of VWF/ factor VIII concentrates
thrombosis/ DVT
chills, HPOTN, HA, edema
viral infection (chance for infection from human blood products)
anaphylaxis
Hemophilia A means patients lack what?
Factor VIII
Hemophilia B means patients lack what?
Factor IX
S/S of hemophilia
palpable ecchymoses
Hemarthroses (joint pain, swelling, decreased ROM)
muscle hemorrhage
hematuria
what labs will be abnormal w/ hemophilia?
prolonged aPTT
reduced factor VIII or IX level
will have normal VW
when should you test for hemophilia?
any male w/ unusual bleeding
what vaccines do patients receiving blood factors need?
Hep A and B
what can be used for mild bleeding in those w/ hemophilia A?
DDAVP
what can you use an an adjunct for prevention of bleeding w/ dental procedures w/ hemophilia.
aminocaproic acid
tranexamic acid
any patient less than 2 w/ hemophilia and no prior bleeding events what do you need to do?
primary prophylaxis
once patients are over 2 w/ hemophilia and don’t have bleeding episodes what do you?
primary prophylaxis
if a patient w/ hemophilia has a bleed what do you need to do?
secondary prophylaxis
what’s the difference b/w primary and secondary prophylaxis
dosing of drugs
what virus is seen in recombinant factor VIII?
parovirus (comes from human cell line)
Does factor IX come from a human cell line?
No
do plasma derived factor or recombinant work better for hemophilia B?
plasma, but better to use recombinant factors because there is less risk
what are some ADRs w/ infusable factors
Chills, fever, HPOTN, edema
thrombosis/ DVT
anaphylaxis
developing antibodies
if developing antibodies more common in hemophilia A or B?
hemophilia A
when should you suspect that the patient has produced IgG antibodies
post-infusion factor levels are lower than predicted
if you can’t overcome the anitbodies produced by person, what must you give?
concentrated activated factors
what is prothrombin compelx concentrates (PCC)
plasma derived
contains Vit K dependent factors
what is used for bleeding episodes or prophylaxis before surgery.
can’t monitor effect w/ labs
Recombinant activated factor VII
what other meds do hemophiliacs need?
pain meds
intraarticular dexamethasone for joint pain
Acquired homeostatic disorder
Due to underlying condition or illness
Inappropriate systemic thrombosis followed by massive bleeding
Disseminated Intravascular Coagulation (DIC)
what are the three things that happen w/ DIC
hypercoagulability –> widespread inflammation
thrombosis (consumes platelets and coags)
bleeding
what are S/S of DIC?
Bleeding/oozing Thrombosis Petichiae/purpura Peripheral cyanosis Gangrene of distal extremities Hemorrhagic bullae
what labs w/ be abnormal w/ DIC (check serially)
Elevated PT and aPTT thrombocytopenia elevated D-dimer and fibrin degradation products (FDP) decreased antithrombin III decreased proteins C and S decreased fibrinogen end organ damage
Tx for DIC (bleedign stage)
platelet infusion
Fresh Frozen Plasma (FFP)
Cryoprecipitate (can be used to replace fibrinogen)
patients with what dz can look like DIC
liver dz (but this has normal fibrinogen where DIC doesn’t)
what drugs do you use for a patient in DIC in thrombotic phase?
heparin or LMWH
when can you consider antifibrinolytics w/ DIC
phase of fibrinolysis/ bleeding
what can you not co-administer w/ antifibrinolytics
heparin
what are the activated factors?
Prothrombin complex concentrates (PCC)
recombinant activated factor VII
