Disorders of Hemostasis

Question 1

What is the difference betwwen primary and secondary Hemostasis?

Answer 1

Primary - injury to formation of platelet plug

Secondary - Activation of plasma coagulation factors to stable clot

Question 2

What is considered abnormal bleeding?

Answer 2

Bleeding that is:

Spontaneous, Excessive, Or delayed

Question 3

What are characteristics of Primary Hemostasis problems?

Answer 3

Mucocutaneous bleeding
hemorrhages in mucosa.
Petechiae - red spots that dont dissappear when pressed
Happen right after trauma.

Question 4

What are characteristics of Secondary Hemostasis problem

Answer 4

Coagulation disorder
Large Hematoma
Delayed bleeding

Question 5

What are tests we can do to see different points in the coagulation cascade?

Answer 5

Peripheral blood smear
Thrombin time
Factor levels
Fibrin/Fibrinogen degradation products (FDP)
Ristocetin-Induced platelet aggregation (RIPA) test

Question 6

What are the various combos of PT and PTT and what do they mean?

Answer 6

PT/PTT
Normal/Normal - platelet Prob - coagulation intact
Normal/Prolonged - prob with intrinsic pathway (hemophilia)
Prolonged/Normal - prob w/ extrinsic pathway
Prolonged/Prolonged - prob with common pathway - very rare

Question 7

What are the degrees of thrombocytopenia?

Answer 7

Mild: 100,000-150
Mod: 50-99,000
Sev:

Question 8

What are the thrombocytopenic emergencies?

Answer 8

Heparin-Induced Thrombocytopenia,
Thrombotic Thrombocytopenic Purpura,
Hemolytic Uremic Syndrome,
Thrombotic microangiopathy

Question 9

What is Bernard-Soulier Syndrome?

Answer 9

Mut GPIb, Big GIANT PLATELETS, VWF cant bind to platelets. superficial bleeds.
Do RIPA test.
Tx: give VIIa, platelets, Desmopressin

Question 10

Glanzmann Thrombasthenia

Answer 10

abnormal GpIIb/IIIa
Normal platelets
cant clump together
Hypoactive agglutination on RIPA
Tx: Recombinant Factor VIIa, Hematopoietic cell transplant

Question 11

Immune Thrombocytopenic Purpura

Answer 11

Dont make platelets and the ones that are made are destroyed
Primary - No other etiology
Secondary - Some other CHRONIC DISEASE
Children and elderly
many are asymptomatic
kids with lots of little petechial hematomas
labs are all normal.
If severe bleeding - hospital
Dx: Recent med, Underlying condition, bleeding, petechiae, sometimes lymph nodes, splenomegaly
Need to rule out VWD, Bernard soullie, other platelet disorders
Tx - nothing to splenectormy, steroids/IVIG
Restrict physical activity.

Question 12

Thrombotic thrombocytopenic Purpura TTP

Answer 12

Platelet disorder with thrombosis
ADAMTS13 protease problem - small vessel prob
Hereditary - mutation
Acquired - AUTOANTIBODy - ORGANS ALL OVER DAMAGED - lungs spared
Supposed to cleave VWF so that it doesnt form clot spontaneously, doesnt work
almost always fatal,
Microangiopathic Hemolytic anemia, thrombocytopenia, fever, renal prob, neuro - rare
Measure ADAMTS13 - if less than 10% then its TTP, if it is higher than 50% - not TTP
Tx - dont wait for results, prompt PLASMA EXCHANGE, Untreated 90% die, treated 80% live

Question 13

Disseminated Intravasculaar Coagulation DIC

Answer 13

Consumption coagulopathy - use up all we have
activation in vessels of coagulation - dont know what, 40-80% mortality
problem with thrombosis then problem with bleeding
Labs - everything is low
DDimers are elevated
schistocytes, like TTP
renal fail, lung fail, liver fail, coma, adrenal fail
Tx: transfusion support of blood or plasma
Acute: Severe bleeding sudddenly, septicemia, snake, acute leukemia
Chronic: exposed to small amounts over time, not a ton of bleeding, but there is a problem with thrombosis more than bleeding.

Question 14

Heparin Induced Thrombocytopenia (HIT)

Answer 14

Caused by heparin - allergy
in 5% of everybody who gets heparin - antibodies against it - see in foot first MORTALITY 20%
More with therapeutic doses, females, surgical patients.
Type I: mild, no need to stop heparin
Type II: later on, antibodies, thrombosis cause problems with all organs, 20% mortality
4 T score - Thrombocytopenia, Timing of platelet fall, Thrombosis, Thrombocytopenia from other causes. If score is high then its likely this. If so - stop giving, support

Question 15

Hemolytic Uremic Syndrome HUS

Answer 15

MAHA - thrombocytopenia, Acute kidney injury
Microangiopathic hemolytic anemia
Primary: atypical
Secondary: infection: Shiga, s pneumo, ecoli
Eat meat with ecoli, toxin binds, blood, damaged vessel, activates platelets, thrombosis and renal damage
Pneumococcal one is more dangerous
Dx: exp to ecoli, labs anemia, thrombocytopenia, shiga toxin or pneumococcal in blood smear
Tx: Support, transfusion, platelet, fluid, dialysis if uremia is bad.

Question 16

Hemophilias

Answer 16

Hemo A is #1 - factor activity greater than 5% is mild, less than 1% severe, not familial,
Big deep hematomas, more significant that dont stop.
Newborn - intracranial, big extracranial bleed
Toddlers bleed alot
Hemarthrosis - in jt, dont poke kids with needle, wt bearing jts
Intracranial Hem - rare but more dangerous
Dx - Hx matches bleeding, Labs (platelet ct/PT NORMAL, prolonged aPTT, Low factors VIII, IX or whatever factor, VWF NORMAL)
Tx - Give Monoclonal antibodies, or recombinant factor, desmopressin if mild/moderate bleeding
Complications - intracranial hemorrhage, stroke, infections, growth prob
Hemo A - Factor VIII def
Hemo B - Factor IX def (christmas disease
Hemo C - Factor XI def (rosenthal syn - jews)
Acquired hemophilia - def bc of autoantibodies
Hemophilia B leyden phenot - Severe hemophilia in childhood, better on PUBERTY

Question 17

Von Willebrand Disease

Answer 17

Most common inherited bleeding disorder
AD - produce AB against VWF
Type I - low quantitative not severe, mild
Type 2 - QUALITATIVE - VWF levels are NORMAL
- 2a - Moderate bleed
- 2b - Moderate Bleed
- 2m - significant bleed
- 2n - AR - mimics hemophilia
Type 3 - Severe quantitativebleed
Labs - Normal platelet, PT, PTT
- Low VWF antigen, VWF activity, Factor VIII activity, VWF multimer/RIPA
Tx: 1 - Desmopressin, 2 - VWF concentrate, adjunctive - fibrinolytic agents, topical hemostatic, new - recombinant VWF

Question 18

Acquired Inhibitors of Coagulopathy

Answer 18

antibodies inhibit factor activity, or increased factor clearance, most common acquired hemophilia A
Look like Hemophilia, prolonged aPTT,
Specific testing - Mixing test, mix plasma with normal plasma and repeat PTT, if it corrects then its a factor deficiency, if it still is prolonged then it is an inhibitor which is just inhibiting the new factors.
Tx - stop bleeding (Desmopressin, factor VIII, prothrombin complex) and try to rid autoantibodies,(glucocorticoids, cyclophosphamide, rituxamab, IVIG)

Question 19

Vitamin K deficiency

Answer 19

Rare, In newborns bleeding from circumcision, etc.
Long term abx, diet etc
Dx: deficient in PROTHROMBIN, FACTORS, VII, IX, X, PROT C

Question 20

Warfarin associated bleeding

Answer 20

tx: Vit K
Serous bleeding: hold warfarin, give VIt K IV, Rapid reversal agents (prothrombin complex)
INR>9 withoug bleeding - Hold warfarin give Vit K oral
INR less than 5, no bleeding - Hold Warfarin, wait, reduce dose
ONLY IF ITS BLEEDING do you give Vit K
If its severe bleed - IV, not severe - oral
Greatest risk of bleeding initially,
other risk factor, older