Coag Disorders for Panada's Sheet Flashcards
What will defective platelets increase in terms of the tests?
bleeding time
What will defective coagulation affect in terms of the tests?
PT or PTT depending on where the defect is
What are two clinical things that are important for thrombophilia?
hypercoagulable state and increased risk for thrombosis
What is arterial thrombosis caused by?
a lsion that exposes the subendothelium to platelets in blood
What is venous thrombosis caused by?
stasis in blood – due to immobility and hypercoagulation
What does an arterial thrombosis look like?
a white clot b/c high platelet component, associated w/ high blood flow
What does a venous thrombosis look like?
a red clot b/c of lower platelets and more fibrin component
Is venous thrombosis more fatal then an arterial one?
no
What is an arterial thrombosis associated w/?
atrial fibrillation, MI>stroke>PAD
How does one treat an arterial thrombosis?
Acute management: Remove clot, stent, aspirin to decrease platelet aggregation, clopidogrel to decrease ADP receptors for aggregation
Chronic – treat underlying disease
What is the Holman Sign?
when one feel pains in the back of the calf during dorsiflexion of the foot b/c DVT clotted veins are painful to stretch
What is the common result of DVT?
pulmonary embolism – SOB is key finding
What is the overall goal for DVT?
keep the clot south of the knee
What should you think of when there is failure of platelet production?
- bone marrow failure
- Megs depression
- c-MPL receptor mutation
What are some causes of increased platelets?
bleeding, splenectomy, inflammatory disease, iron deficiency
What is the clinical presentation for thrombocytopenia?
- spontaneous skin purpura
- mucosal hemorrhage
- excess bleeding post-trauma
What is ITP?
IgG Abs targeted at platelets causing them to be removed by RES system
What can cause ITP?
drug induced —w/ heparin and PF4 complex formation
What is the clinical presentation for ITP?
young women, thrombocytopenia, but no neutropenia or anemia, lots of PETEICHIA!
What is the treatment for ITP?
- common w/ URI in kids – no treatment
2. adults - give prednisone, IVIg, TPO agonist to make more platelets, and splenectomy
What is TTP?
- congenital – absence/defective ADAMTS13
- Acquired - auto-Ab against ADAMTS13
- large vWF– binds platelets – occlusion due to aggregation –> microangiopathic anemia
What is ADAMTS13?
metalloprotease that cleaves large vWF
What is the treatment for TTP?
- plasma exchange, FFP, steriods, rituximab
2. NEVER GIVE PLATELETS
What is the presentation for TTP?
young women, thrombocytopenia, MAHA, neurological symptoms, renal insufficiency, fever
What is seen in the PS for TTP?
schistocytes
What can a DDx be made w/ for TTP?
- thromboctyopenia
- schistocytes/MAHA
- increased LDH
- increased indirect bilirubin
What causes DIC?
infection, malignancies, OB complications
What is the key problem in DIC?
Excess thrombin production –> excess fibrin deposition –> clot formation –> MAHA
What is the Rx for DIC?
- treat underlying cause
2. platelets, FFP, antithrombin 3, activated protein C
What is the clinical presentation for DIC?
thromboctopenia, purpura, peripheral gangrene
What is the DDx for DIC?
- low platelets
- low fibrinogen
- low clotting factors
- high D-Dimer —> KEY TEST FOR DIC
What is HHT?
AD, occurs in arterioles/small veins, and platelets are normal
What is the clinical presentation for HHT?
easy bruising, spontaneous bleeding from small vessels, mucuosal bleeding
What will you commonly see in pts w/ HHT?
bleeding on edge of tongue, inside noses. Look for redspots
What is senile purpura?
age-associated atrophy of vasculature so bruising at commonly injured sites
What is Ehlers Danlos Syndrome?
defective synthesis of connective tissue
What is Henoch Schoeiein Purpura?
IgA mediated vasculitis –> you can feel the purpura!
What is bernard soulier?
deficient in GpIb
What is Glanzman’s Thrombasthenia>
deficient in GpIIb/IIIa
What are acquired causes of platelet disease?
- drugs
2. liver/renal metabolic failure
What is the treatment for acquired autoimmune thrombocytopenia due to platelet transfusions?
No treatment option left so be careful
What is Hemophilia A?
absence/ lower plasma factor 8, x-linked recessive
What is hemophilia B?
absence/ lower plasma factor 9, x-linked recessive
What is the clinical presentation for hemophlia A and B?
deep bleeding, bleeding into joints, fusion of joints
What is the DDx for hemophilia?
- long PTTS and normal PT
2. check for inhibitors and then confirm w/ factor assay
What is the Rx for hemophilia?
- EDUCATION
- local - pressure, topical thrombin
- system - desmopressin (increase vWF), E-aminocaproic acid (inhibits fibrinolysis)
What is vWF Disease?
absent/reduced vWF, AD
What are the two functions of vWF?
platelet binding and 8 carrier
What is the clinical presentation for vWF disease?
bruising, mucosal bleeding, common when young women begin menstruating
What is the DDx for vWF?
- long PTT, normal PT
- Measure vWF
- Longer bleeding time
- Ristoceitin Test
What is the Rx for vWF?
- DDAVP and E-aminocaproic acid
What is the DDx for factor 5 mutation?
- add activated protein C and observe thrombin time. If mutation is present then PT/PTT time will be normal.
- Screen for mutation
What is the Rx for factor 5 mutation?
if no thrombosis, don’t start on anticoagulant therapy unless high risk situation (long flights)
What is the most common inherited thrombophilia?
factor 5 leiden mutation
What presents w/ reurring venous thrombosis starting in early adult life?
inherited antithrombin 3 deficiency
What is inherited antithrombin 3 deficiency?
- AD
2. deficiency in antithrombin 3 so tissue factors aren’t activated –> hypercoaguable state
What is the test for an antithrombin 3 deficiency?
inject heparin and look at PTT, should be no change in PTT b/c heparin has nothing to bind to
What is the Rx for antithrombin 3 deficiency?
antithrombin replacement
What is inherited Protein C and S deficiency?
- AD, hypercoaguable state
What is the presenation for protein C and S deficiency?
skin necrosis, thrombosis
What is the Rx for protein c/s deficiency?
activated protein C concentrate for replacement
What is the inherited prothrombin gene mutation?
G20210A,
- Sustained generation of thombin
- increased thrombosis
- decreased fibrinolysis b/c activated thrombin stimulates secretion of anti-tPA
What is the DDx for the prothrombin gene mutation?
- analyze genetic mutation
2. analyze levels of clotting factors and PT/PTT
What is inherited hyperhomocysteinemia?
Bad MTHFR -? no remethylation of THF –> no substrate for the conversion of homocysteine to methionine
- — bulidup of homocysteine = thrombosis
Who is hyperhomocysteinemia common in?
look for unusual thrombosis or CVD in children
What are some causes of acquired thrombophilia?
- ESTROGEN - increased risk for DVT
- BIRTH CONTROL - increased B-thromboglobulin and decreased antithrombin
- pregnancy
- immobilization
- trauma
- advanced ago
- antiphospholip
- cancer
- ET
- nephrotic syndrome
What is the anti-phospolipid syndrome?
Abs directed at phospholipid components
- Anti-cardiolipin
- antiPS
- associated proteins: B2GP and thrombin
- common finiding is lupus anticoagulant
What is the clinical presentation for anti-phospholipid syndrome?
- thrombosis
- fetal loss in 2nd semester
- dermatological issues
- blurred vision
- stroke
- Thrombosis w/ anticoagulant lupus but no SLE diagnosis
What is the DDx for anti-phospholipid syndrome?
- high PTT is not corrected, shows inhibitor of coagulation, no deficiency in clotting factor
- corrected in hexagonal phase b/c it competes for binding of lupus anticoagulant
- Russell Viper Venom Test
What does the russell viper venom test test for?
factors 10 and 5
What is the HIT syndrome?
- autoAb develops for heparin-PF4 complex
- Ab and complex binds to platletes via platelets Fc receptor
- causes platelet aggregation and activation
What is the clinical presentation of HIT?
- thrombosis but not bleeding
What will labs show for HIT?
- thrombosis – aggregation of platelets
2. thrombocytopenia – increased use of platelets
What is the DDx for HIT?
look for anti-Heparin/PF4 Ab via ELISA
What is the Rx for HIT?
take pt off heparine, give DTI
- don’t give DTI via IV if kidney disease
What is coumarin/coumadin induced necrosis?
- Warfarin therapy has immediate effects in liver but delayed systemic effects b/c circulating clotting factors must be cleared
- systemic effect take 1 wek
- anti-coagulation factors, PC and PS, have shorter half lives. Therefore, inhibition of PC/PS formation generates clots
- Results in tissue necrosis
What are effects of coumadin?
tissue necrosis of skin/fatty areas
What is the Rx for warfarin induced necrosis?
- remove warfarin
- administer Vit K
- administer prothrombin complex (replace all Vit K factors including PC/PS
- FFP - replace all clotting factors
- recombinant 7a
How do you prevent wafarin induced necrosis?
give heparin and warfarin at same time and wean off heparin
