Disorders of Hemostasis Flashcards
What is the difference betwwen primary and secondary Hemostasis?
Primary - injury to formation of platelet plug
Secondary - Activation of plasma coagulation factors to stable clot
What is considered abnormal bleeding?
Bleeding that is:
Spontaneous, Excessive, Or delayed
What are characteristics of Primary Hemostasis problems?
Mucocutaneous bleeding
hemorrhages in mucosa.
Petechiae - red spots that dont dissappear when pressed
Happen right after trauma.
What are characteristics of Secondary Hemostasis problem
Coagulation disorder
Large Hematoma
Delayed bleeding
What are tests we can do to see different points in the coagulation cascade?
Peripheral blood smear
Thrombin time
Factor levels
Fibrin/Fibrinogen degradation products (FDP)
Ristocetin-Induced platelet aggregation (RIPA) test
What are the various combos of PT and PTT and what do they mean?
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
What are the degrees of thrombocytopenia?
Mild: 100,000-150
Mod: 50-99,000
Sev:
What are the thrombocytopenic emergencies?
Heparin-Induced Thrombocytopenia,
Thrombotic Thrombocytopenic Purpura,
Hemolytic Uremic Syndrome,
Thrombotic microangiopathy
What is Bernard-Soulier Syndrome?
Mut GPIb, Big GIANT PLATELETS, VWF cant bind to platelets. superficial bleeds.
Do RIPA test.
Tx: give VIIa, platelets, Desmopressin
Glanzmann Thrombasthenia
abnormal GpIIb/IIIa Normal platelets cant clump together Hypoactive agglutination on RIPA Tx: Recombinant Factor VIIa, Hematopoietic cell transplant
Immune Thrombocytopenic Purpura
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.
Thrombotic thrombocytopenic Purpura TTP
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
Disseminated Intravasculaar Coagulation DIC
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.
Heparin Induced Thrombocytopenia (HIT)
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
Hemolytic Uremic Syndrome HUS
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.
Hemophilias
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
Von Willebrand Disease
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
Acquired Inhibitors of Coagulopathy
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)
Vitamin K deficiency
Rare, In newborns bleeding from circumcision, etc.
Long term abx, diet etc
Dx: deficient in PROTHROMBIN, FACTORS, VII, IX, X, PROT C
Warfarin associated bleeding
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
