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Hematology > Bleeding, Thromotic, and Fibrinolytic Disorders > Flashcards

Bleeding, Thromotic, and Fibrinolytic Disorders Flashcards

1
Q

What are the two main functions of von Willebrand factor?

A

Binds platelets to form the initial platelet plug. Binds with Factor VIII to prolong it’s half life

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2
Q

What is the most common inherited bleeding disorder?

A

von Willebrand, autosomal dominant

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3
Q

Describe the different types of von Willebrand disease

A

Type 1-Quantitative abnormality of vWF, most common. Type 2-Qualitative abnormality, Decreased binding to factor VIII and platelets, resembles hemophilia A. Type 3 -Rare, Undetectable levels of vWF and severe bleeding in infancy and childhood

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4
Q

What are symptoms of von Willebrand disease?

A

Nosebleeds > 10 min in childhood. Lifelong easy bruising (associated hematoma). Bleeding following dental extractions or other surgery. Heavy menstrual bleeding or post partum

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5
Q

What laboratory tests should be done for von Willebrand disease?

A

Plasma vWF antigen, Plasma vWF activity (vWF:Rco and vWF collagen), Factor VIII activity. PT normal. PTT normal or prolonged depending on the Factor VIII activity

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6
Q

What are treatment options for von Willebrand disease?

A

DDAVP (minor bleeding), vWF concentrate (major bleeding or for type 2 and 3). Avoid aspirin/NSAIDs

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7
Q

What is the pathophysiology behind disseminated intravascular coagulation?

A

Massive release of tissue factor. Tissue factor sets the coagulation system in place. Coagulation occurs. Clotting factors and inhibitors are consumed. Clots further trap circulating platelets leading to ischemia

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8
Q

What happens as a result of excess thrombin in DIC?

A

activates plasmin resulting in fibrinolysis. This breakdown of clots = fibrin degradation products which have further anticoagulant properties. Plasmin also activates the complement and kinin systems = shock

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9
Q

What are common causes of DIC?

A

massive tissue injury, obstetric complications, sepsis, snake bite, cancer

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10
Q

What lab results are associated with DIC?

A

Thrombocytopenia. Prolongation of PT and PTT. Low fibrinogen. Increased levels of fibrinogen degradation products (d-Dimer). Schistocytes (helmet cells)

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11
Q

What are treatment options for DIC?

A

Anticoagulants only to prevent imminent death. Platelets or coagulation factor replacement in the case of plts < 50K and serious bleeding. Fresh frozen plasma or cryoprecipitate to keep fibrinogen > 100 mg/dL if significantly elevated PT/INR

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12
Q

What is the pathophysiology behind hypercoagulable states?

A

endothelial damage and inflammation, elevated platelet levels

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13
Q

What conditions accelerate the activity of the clotting system?

A

Pregnancy. Oral contraceptives. Postsurgical state. Malignancy (renal cell carcinoma). Hereditary clotting disorders

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14
Q

What are risk factors for abnormal clotting?

A

Stasis/Immobility (DVT). Low cardiac output (CHF). Obesity. Sleep apnea

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15
Q

What are the hereditary clotting disorders?

A

Protein C, Protein S, Antithrombin III, Factor V Leiden, Antiphospholipid antibody

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16
Q

What is the fxn of protein C?

A

inactivates Factors V and VIII thereby inhibiting anticoagulation. A deficiency of Protein C leads to prolonged action of Factors V and VIII leading to excessive clotting

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17
Q

What are symptoms of protein C deficiency?

A

Thrombosis, Deep vein thrombosis, Pulmonary embolism, Thrombophlebitis

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18
Q

How should you work up a suspected protein C deficiency?

A

Protein C, PTT, PT, Thrombin time, Bleeding time,

Medical and family history

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19
Q

What are treatment options for protein C deficiency, protein S deficiency, antithrombin III deficiency, factor V Leiden?

A

Treat with anticoagulants if at high risk for clotting such as surgery or hospitalization. Chronic anticoagulation if history of thrombosis

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20
Q

What is the fxn of protein S?

A

needed for proper function of Protein C. A deficiency in Protein S results in diminished ability of Protein C to inactivate Factors V and VIII resulting in excessive clotting

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21
Q

What is antithrombin III deficiency?

A

antithrombin is a major inhibitor of thrombin and other clotting Factors including X and IX. deficiency results in morbidity/mortality. Recurrent venous thrombosis, pulmonary embolism and repetitive intrauterine fetal death. onset is 15-35 years old

22
Q

How should you work up a suspected antithrombin III deficiency?

A

Antithrombin-heparin cofactor assay is the best screening test for AT deficiency (Measures functional AT activity). Standard coagulation tests should be normal (PT, PTT)

23
Q

What is factor V Leiden?

A

Most common genetic disorder to cause DVT. Lack of Factor V Leiden decreases the anticoagulant activity of the activated protein C

24
Q

What are symptoms of factor V Leiden?

A

Thrombophlebitis, Deep vein thrombosis, Pulmonary embolism

25
Q

What is antiphospholipid antibody?

A

Autoimmune hypercoagulable state caused by antiphospholipid antibodies. Antibodies lead to arterial and venous clot formation (stroke, DVT)

26
Q

What are complications of antiphospholipid antibodies?

A

Pregnancy complications: miscarriage, stillbirth, preterm delivery, severe eclampsia. End organ damage

27
Q

How is the diagnosis of antiphospholipid antibody made?

A

Anti-cardiolipin antibodies IgG and IgM OR Lupus anticoagulant OR Anti-beta2 glycoprotein I IgG and/or IgM. Plus history of arterial or venous thrombosis

28
Q

What are treatment options for antiphospholipid antibody disease?

A

Aspirin, Warfarin or other oral long term anticoagulants

29
Q

What are some types of platelet disorders?

A

Immune thrombocytopenia, thrombotic thrombocytopenia, hemolytic uremic syndrome

30
Q

What are the causes of Immune thrombocytopenia?

A

Usually idiopathic
Secondary causes: autoimmune disorders like lupus ect, medications (sulfonamides, thiazides, cimetidine, heparin), viral infections (HIV, hepatitis C)

31
Q

How is immune thrombocytopenia different in children than adults?

A

Commonly occurs in childhood after a viral infection with spontaneous resolution, tends to be chronic in adults with females more common

32
Q

What is the pathophysiology of Immune thrombocytopenia?

A

antibodies bind to platelets-destruction of platelets, inadequate preduction of platelets

33
Q

What are the clinical manifestations of ITP?

A

Mucocutaneous bleeding (blood blisters in mouth), Petechiae, purpura, Spontaneous bruising,Nosebleeds, gingival bleeding, Retinal hemorrhage, Excessive menstrual bleeding, Melena (from upper GI bleed), hematuria

34
Q

What is needed for diagnosis of ITP?

A

Thrombocytopenia. Normal RBC morphology. Prolonged bleeding time. +/- anemia. PT/PTT are normal. Bone marrow biopsy-Normal or increased number of megakaryocytes

35
Q

What are treatment options for ITP?

A

Treat w/prednisone or IVIG if platelet counts are less then 20-30,000 or if significant bleeding. prednisone or dexamethasone. rituximab or TPO receptor agonist. platelet transfusion if needed. BMT or chemo in severe cases.

36
Q

What is thrombotic thrombocytopenic purpura?

A

inappropriate platelet aggregation that leads to destruction of RBCs. Antibodies against ADAMTS-13 which is responsible for cleaving large vWF molecules into smaller pieces

37
Q

What are causes of TTP?

A

Primary causes: autoimmune. Secondary causes: Cancer, BMT, Pregnancy, Meds: acyclovir, Quinine, ticlodipine, clopidogrel, prasugrel, cyclosporine, mitomycin, tacrolimus, interferon alpha), HIV

38
Q

Where does organ damage occur in TTP due to microscopic clotting?

A

the kidneys and brain

39
Q

What are the 5 main characteristics of TTP?

A

Thrombocytopenia. Microangiopathic hemolytic anemia. Neurologic symptoms. Kidney failure. Fever

40
Q

What laboratory abnormalities are seen with TTP?

A

RBC fragmentation, elevated indirect bilirubin, decreased serun haptoglobin, elevated LDH

41
Q

What is the main treatment of TTP?

A

plasma exchange

42
Q

What is hemolytic uremic syndrome?

A

caused by endothelial damage secondary to bacterial toxins (e.coli, shigella) and platelet aggregation

43
Q

What are the main signs of HUS?

A

Microangiopathic hemolytic anemia, Acute kidney injury and renal failure, Thrombocytopenia, bloody diarrhea (distinguishes btw TTP)

44
Q

What are treatment options of HUS?

A

Transfuse RBCs and platelets if needed, Dialysis if symptomatic uremia, Nutritional and electrolyte support, may consider plasma exchange

45
Q

What is Henoch-Schonelin purpura?

A

IgA vasculitis occurs in children 3-15yrs old and can be triggered by strep upper respiratory infection.

46
Q

What are themajor symptoms associated with HSP?

A

Palpable purpura, Arthritis/arthralgias, Abdominal pain, Renal disease, Too much IgA. no thrombocytopenia (differentiates btw ITP/TTP)

47
Q

What are treatment options for HSP?

A

NSAIDs, glucocorticoids

48
Q

Describe the common types of hemophila

A

hemophilia A: most common, factor VIII deficiency. Hemophilia B: factor IX deficiency. Hemophilia C: factor XI deficiency, rare

49
Q

What characterizes the bleeding of hemophila?

A

spontaneous hemarthrosis that can cause deformity long-term. At risk for spontaneous intracerebral hemorrhage. Most common bleeding sites are the Joints, Skin, muscle GU, GI

50
Q

What are treatment options for hemophila?

A

Replace the deficient factor usually 3 times per week. Avoid aspirin/NSAIDS. Cryoprecipitate or DDAVP (desmopressin) if factors unavailable.

51
Q

What are diagnostics of hemophila?

A

PTT prolonged. other platelet tests normal. Diagnosis is confirmed by decreased levels of Factor VIII, IX or XI

Hematology (16 decks)

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