Term
| What is the role of Thrombomodulin, bound to Endothelial cells? |
|
Definition
| Activates Protein-C to inactivate factor V and VIII and inhibit over-coagulation |
|
|
Term
| vWF and Glycoprotein Ib will cause the platelets to do whay? |
|
Definition
| Adhere to the endothelial surface (it interacts with vWF) |
|
|
Term
| Glycoprotein IIb and IIIa and Fibrinogen will cause platelets to do what? |
|
Definition
| Aggregate rather than adhere |
|
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Term
| What is the role of the lab test that uses Ristocetin? |
|
Definition
| Ristocetin reacts with vWF and Glycoprotein Ib to determine whether or not there is an Adherance issue with platelets |
|
|
Term
| What is Bernard-Soulier Syndrome? |
|
Definition
| Abnormal platelet adherance disease due to defective GPIb/IX platelet membrane receptor which impairs binding to vWF |
|
|
Term
| What is the clinical finding on blood smear of Bernard-Soulier Syndrome? |
|
Definition
| less platelets but Giant platelets |
|
|
Term
| What is Glanzmann's Thrombasthenia? |
|
Definition
| Deficiency of GPIIb/IIIA leading to Abnormal Aggregation of platelets |
|
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Term
| What count is there a risk from: Major surgery, Minor surgery/bruising, Spontaneous petechiae, Slinically significant bleeding |
|
Definition
| 50-80k is risky for major surgery, 20-50k bruises/minor surgery, <20k spontaneous petechiae, Significant bleeding <10k |
|
|
Term
| Pseudothrombocytopenia can occur under what circumstances? |
|
Definition
| When specimens are collected in EDTA which can cause agglutination of platelets due to antibodies |
|
|
Term
| What hormone Regulates thrombopoiesis and where is it produced? |
|
Definition
| Thrombopoietin (TPO) produced in the Kidney and Liver |
|
|
Term
| What two drugs can be given to act like Thrombopoietin? |
|
Definition
| Romiplostim and Eltrombopag |
|
|
Term
| What all can cause Thrombocytopenia? |
|
Definition
| Immune mediated disorders, Drugs like heparin, Thrombotic Thrombocytopenic Purpura, DIC, Obstetrical complications |
|
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Term
| The spleen normally holds about 1/3 total platelet pool, and can cause Thrombocytopenia from spelenic sequestration due to association with what other disorder? |
|
Definition
| Portal Hypertension (causes splenomegally) |
|
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Term
| How can prednisone be used to remove antibody-coated platelets? |
|
Definition
| Activates splenic macrophages to take out antibody coated platelets and inhibits IgE production |
|
|
Term
| What is the cause of Idiopathic Thrombocytopenic Purpura (ITP)? |
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Definition
| IgG autoantibodies against platelet membrane glycoproteins with subsequent destruction of these Ab-coated platelets in the RES |
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Term
| What type of condition may be diagnosed before or after onset of Idiopathic Thrombocytopenic Purpura (ITP)? |
|
Definition
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|
Term
| Acute ITP is Triggered by what, is most often found in what age group and what kind of therapy does it call for? |
|
Definition
| Triggered by viral infection or allergic reaction; Children; no therapy needed |
|
|
Term
| Chronic ITP is most common in what age group, what is the clinical presentation, and what can secondary cases be due to? |
|
Definition
| Adults; NO splenomegaly, secondary to SLE (Lupus), HIV, HCV |
|
|
Term
| What are the key lab findings for diagnosing ITP? |
|
Definition
| Normal Pt, PTT; ruled out HIV and HCV antibodies, and Megakaryocyte Hyperplasia is noted |
|
|
Term
| What is primary treatment for ITP? |
|
Definition
| Prednisone empirically followed by Splenectomy if necessary |
|
|
Term
| If rapid treatment to ITP is desired, what can be administered? |
|
Definition
|
|
Term
| What is the Classic Pentad of Thrombotic Thrombocytopenic Purpura (TTP)? |
|
Definition
| 1) Microangiopathic Hemolytic Anemia (MAHA), 2) Thrombocytopenia, 3) Fever, 4) Renal abnormalities, 5) CNS issues |
|
|
Term
| Thrombotic Thrombocytopenic Purpura is due to a deficiency of what? |
|
Definition
| a vWF cleaving metalloprotease called *ADAMTS13 |
|
|
Term
| As a result of deficiency of the vWF cleaving metalloprotease ADAMTS13 in TTP, what happens when the excess vWF is released? |
|
Definition
| They induce platelet adhesion and aggregation which results in disseminated platelet thrombi in microvasculature (MAHA) |
|
|
Term
| Peripheral smear of TTP will show what? |
|
Definition
| Thrombocytopenia, high Retic count, and Schistocytes from MAHA |
|
|
Term
| What are the results of coagulation tests in TTP? |
|
Definition
|
|
Term
| What is the confirmatory test for TTP? |
|
Definition
|
|
Term
|
Definition
| Plasma Exchange to remove antibody against ADAMTS13 |
|
|
Term
| What is the fatality of TTP and what should you avoid administering to TTP patients? |
|
Definition
| 95% mortality BEFORE plasma exchange; you should avoid platelet transfusions |
|
|
Term
| What is the cause of Hemolytic Uremia Syndrome? |
|
Definition
| E. coli O157:H7 and Shigella (Shiga toxin) |
|
|
Term
| What can be noted histologically in the glomeruli of Hemolytic-Uremic patients and what does it result in? |
|
Definition
| Fibrin-plated thrombi; results in renal failure |
|
|
Term
| What is the treatment of Hemolytic Uremia Syndrome in adults? |
|
Definition
| Treating with antibiotics makes the syndrome worse (releases more toxin), so plasma exchange is best bet |
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|
Term
| Factor VIII deficiency is known as what an what kind of bleeding show? |
|
Definition
| Hemophilia A (DEEP tissue bleeding) |
|
|
Term
| Inheritance of Hemophilia A |
|
Definition
|
|
Term
| Lab diagnosis of Hemophilia? |
|
Definition
| Normal platelets, bleeding time, PT time, but Prolonged PTT |
|
|
Term
| Factor IX deficiency is known as... |
|
Definition
| Hemophilia B (1/5 as common as Hemophilia A) |
|
|
Term
| Type I von Willebrand's disease results in what? |
|
Definition
|
|
Term
| Acceptable treatment for Type I von Willebrand's disease is what and why? |
|
Definition
| Desmopressin (DDAVP); elevates vWF and Factor VIII from endothelial cells |
|
|
Term
| What all does vWF attach to? |
|
Definition
| Collagen, Platelets, and *Factor VIII* |
|
|
Term
| How does vWF manifest in form? |
|
Definition
| as a series of multimers, with larger forms that mediate platelet adhesion and are reduced in size (kept in check) by ADAMTS12 |
|
|
Term
| What is the most common inherited coagulopathy and what type of inheritance is it? |
|
Definition
| von Willebrand's Disease; autosomal dominant |
|
|
Term
| What are the characteristics of vWF disease? |
|
Definition
|
|
Term
| What are the Lab findings for von Willebrand's disease in terms of PTT, VIII, vWF antigen, and Ristocetin Co-factor? |
|
Definition
| Prolonged PTT, low factor VIII and vWF antigen, Low Ristocetin Co-factor |
|
|
Term
| What are the two treatments for von Willebrand's disease? |
|
Definition
| Desmopressin (DDAVP) to induce the release of vWF and VIII from endothelial cells OR Humate P |
|
|
Term
| What is the cause of Disseminated Intravascular Coagulopathy (DIC)? |
|
Definition
| Massive activation of both the coagulation cascade and the fibrinolytic pathway in the circulation |
|
|
Term
| What is the result of the massive activation of the coagulation/fibrinolytic cascade in DIC? |
|
Definition
| Thrombocytopenia, prolonged PT, PTT, hypofibrinogenemia, increased D-Dimer |
|
|
Term
| What microbe is a common cause of DIC? |
|
Definition
|
|
Term
| Which two factors fall the fastest in DIC? |
|
Definition
|
|
Term
| What can DIC cause in the extremities and organs? |
|
Definition
| Gangrene in extremities and Organ Failure |
|
|
Term
| What does treatment of Consumptive Coagulopathy (like DIH) involve? |
|
Definition
| Treatment of the underlying disorder |
|
|
Term
| Liver disease will cause a decrease in all factors except... |
|
Definition
| Factor VIII (made in endothelial cells as well) |
|
|
Term
| What is the most common inherited Thrombophilia? |
|
Definition
|
|
Term
| What two factors have the shortest half life and what is the significance? |
|
Definition
| Factor VII (4-6 hours) and Protein C (8 hours); they can regenerate relatively quickly after warfarin administration |
|
|
Term
| What are Virchow's Triad leading to clots |
|
Definition
| 1) Venous stasis, 2) Injury to vessel wall, 3) Hypercoagulability (such as Factor V Leiden mutation) |
|
|
Term
| Risk factors for Venous Thrombosis Embolism |
|
Definition
| age of 40+, Obesity, previous VTEs, impaired cardiac output |
|
|
Term
| How is a Deep Vein Thrombosis (DVT) diagnosed? |
|
Definition
| Duplex Doppler Ultrasound and D-dimer |
|
|
Term
|
Definition
| Tendency to venous thromboembolism |
|
|
Term
| What are some causes of Inherited Thrombophilia (clot formations) |
|
Definition
| Deficiency of Protein C, S, Antithrombin, Factor V Leiden, and Prothrombin 20210 mutation |
|
|
Term
| What are the indications for pursuing a work up for Inherited Thrombophilia? |
|
Definition
| DVT at a young age (under 40) and a family history of Thromboembolic disease |
|
|
Term
| What lab tests should be run for Thrombophilia? |
|
Definition
| Factor V Leiden, Prothrombin 20210, Protein C/S |
|
|
Term
| A patient with protein C deficiency who is put on Warfarin is at risk for what? |
|
Definition
| Warfarin Induced Skin Necrosis |
|
|
Term
| What is the clinical picture for Antiphospholipid Antibody (Lupus Anticoagulation)? |
|
Definition
| Autoimmune condition with associated vascular thrombus and/or recurrent pregnancy loss (remember phospholipid prevents clot formation) |
|
|
Term
| What happens to PTT and with a 1:1 mix in Antiphospholipid Antibody condition (Lupus Anticoagulation)? |
|
Definition
| Prolonged PTT that fails to correct with 1:1 mix (due to the antibodies present) |
|
|
Term
| What causes the prolonged PTT in vitro in Antiphospholipid Antibody (Lupus Anticoagulation)? |
|
Definition
| IgG or IgM that binds to phospholipid associated proteins |
|
|
Term
| What happens to the Dilute Russell Viper Venom Time and Anticardiolipin antibody in Antiphospholipid Antibody disease (Lupus Anticoagulation)? |
|
Definition
| Prolonged Dilute Ressell Viper venom time and positive Anticardiolipin antibody |
|
|
Term
| What is the treatment for Lupus Anticoagulant? |
|
Definition
| Long term low molecular weight Heparin (NOT warfarin due to teratogen) |
|
|
Term
| What is the cause of Heparin-induced Thrombocytopenia? |
|
Definition
| Antibodies that develop against Platelet Factor 4 and Heparin |
|
|
Term
| What is the progression and diagnostic steps to be taken for Heparin-induced Thrombocytopenia? |
|
Definition
| Platelet count drops with no other cause of thrombocytopenia identified; Order lab tests to identify HIT but discontinue heparin immediately |
|
|
Term
| What synthetic peptide inhibitor can be given to patients with Heparin-induced Thrombocytopenia? |
|
Definition
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