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Hematology > Bleeding, Thrombotic and Fibrinolytic Disorders > Flashcards

Bleeding, Thrombotic and Fibrinolytic Disorders Flashcards

1
Q

What are the 3 platetelet disorders?

A

Immune Thrombocytopenia
Thrombotic thrombocytopenia purpura
Hemolytic uremic syndrome

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2
Q

What are the hallmark signs of a platelet disorder?

A

petechiae and purpura (should maintain the color if pressed on)

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3
Q

What are the causes of ITP?

4

A 
Primary: idiopathic
Secondary: 
Autoimmune
Medications
Viral infections
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4
Q

What medications can cause ITP?

4

A

sulfonamides,
thiazides,
cimetidine,
Heparin

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5
Q

What are examples of infections that can cause ITP?

2

A

HIV, Hepatitis C

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6
Q

What is ITP in children often provoked by?

A

viral illness

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7
Q

Is ITP better in adults or children?

A

Better and more transiet in children
Onset 2-21 days after viral infection with spontaneous resolution

Adults are often more chronic

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8
Q

Describe the pathology of ITP?

3

A

Antibodies bind platelets

Leads to destruction of platelets

Inadequate production of platelets

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9
Q

The disease process of ITP exlplained again?

4

A

1) Increased platelet destruction is caused by autoantibody binding to platelets.
2) Increased endogenous thrombopoietin (TPO) clearance results in reduced levels.
3) Megakaryocytes may be damaged by antibodies, making them less productive than normal.
4) Suboptimal platelet production results from damaged megakaryocytes and reduced TPO levels.

  1. antibodiies tag platelets for destruction by the spleen
  2. Becuase of destruciton of platelts, TPO clearence is increased (more is excreted)
  3. megakaryocytes are damaged
  4. overall, low platelet levels, damgaed megakaryocytes and low TPO levels
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10
Q

Clinical manifestations of ITP?

8

A
  1. Mucocutaneous bleeding (blood blisters in mouth)
  2. Petechiae, purpura
  3. Spontaneous bruising
  4. Nosebleeds
  5. gingival bleeding
  6. Retinal hemorrhage
  7. Excessive menstrual bleeding
  8. Melena, hematuria
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11
Q

Labs and procedures for diagnosing ITP and exclusing other things
6

2 positive tests
2 tests that may indicate ITP
2 tests that should be normal if they have ITP

A
  1. Thrombocytopenia
  2. Prolonged bleeding time (after a cut)
  3. Normal or increased number of megakaryocytes
  4. +/- anemia
  5. PT/PTT are normal
  6. Bone marrow biopsy
    - Normal RBC morphology
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12
Q

When should we treat ITP?

2

A

Don’t treat unless platelet counts are less than 20,000-30,000 or if there is significant bleeding

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13
Q

What should we treat ITP with if we are going to treat it?

3

A
  1. Oral steroids with prednisone or dexamethasone
  2. (if its not working then give IVIG)
  3. May give platelet transfusion if needed
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14
Q

If a patient fails to respond to oral steriods for ITP treatment what are the other options?
5

A
  1. IVIG or anti-D immune globulin
  2. Rituximab (antibody against CD20)
  3. Thrombopoietin receptor agonist
  4. Splenectomy
  5. For severe cases bone marrow transplant or various chemotherapy agents
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15
Q

What is TTP?

A

disorder of inappropriate platelet aggregation that leads to destruction of RBCs.

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16
Q

What is the function of ADAMT13?

A

responsible for cleaving large vWF molecules into smaller peices

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17
Q

Pathgology behind TTP?

3

A
  1. Antibodies against ADAMTS-13 which is responsible for cleaving large vWF molecules into smaller pieces
  2. Extensive platelet aggregation and fibrin bridging
  3. Shear force on RBCs lead to destruction
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18
Q

TTP is characterized by what three things?

A
  1. Thrombocytopenia
  2. Inappropriate platelet aggregation and formation of fibrin
  3. Anemia from the RBCs lysing secondary to the shear forces encountered due to the extensive microemboli
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19
Q

Primary causes of TTP?

A

Autoimmune

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20
Q

Secondary causes of TTP?

5

A 
Cancer
Bone marrow transplant
Pregnancy
Meds
HIV
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21
Q

Meds that can cause TTP?

9

A 
acyclovir, 
Quinine, 
ticlodipine, 
clopidogrel, 
prasugrel, 
cyclosporine, 
mitomycin, 
tacrolimus, 
interferon alpha

Antiviral drugs (acyclovir)
Quinine
Oxymorphone
Platelet aggregation inhibitors (ticlopidine, clopidogrel, and prasugrel)
Immunosuppressants (cyclosporine, mitomycin, tacrolimus/FK506, interferon-α)
Hormone altering drugs (estrogens, contraceptives, hormone replacement therapy)[12

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22
Q

Where does organ damage primarily occur in TTP?

Why does it cause organ damage?

A

kidneys and the brain

Microscopic clotting leads to end organ damage due to ischemia

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23
Q

What are the five main characteristic of TTP?

A
  1. Thrombocytopenia
  2. Microangiopathic hemolytic anemia
  3. Neurologic symptoms
  4. Kidney failure (may be mild or absent)
  5. Fever (75% of patients)
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24
Q

Symptoms caused by secondary effecs of underlying microvascular clotting disorder?
4

A
  1. Malaise
  2. Diarrhea
  3. Thrombocytopenia = bruising, bleeding
  4. Microvascular clotting leads to organ damage = kidney failure, neurologic symptoms and others
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25
Q

What lab abnormalities would we see in TTP?

5

A
  1. Microangiopathic hemolytic anemia (prominent rbc fragmentation)
  2. Elevated indirect bilirubin
  3. Decreased serum haptoglobin
  4. Severely elevated LDH (lactate dehydrogenase)
  5. Anemia
  6. High indirect bili
  7. Low serum haptoglobin
  8. REALLY high LDH
  9. presence of shishtocytes
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26
Q

Treatment of TTP?

What is the main treatment and what is essential in treating the disease successfully?

A

Main treatment is plasma exchange

Early diagnosis and treatment is essential

Without treatment 90% of patients die

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27
Q

What is hemolytic uremic syndrome most commonly causes by?

Can be caused by other things what are they?
10

A

E. Coli

E. Coli 0157:H7
Shigella dysenteriae
Streptococcus pneumonia
Quinine
Chemotherapy drugs
Cyclosporine
Anti-platelet medications
Pregnancy
HIV
Genetic
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28
Q

What kind of bacteria causes hemolytic uremic syndrome?

A

gram negative bacteria because of exotoxin release from these organims

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29
Q

WHat is HUS secondarily caused by?

A

enodthelial damage

  • Widespread injury throughout the body
  • sevre inflammation response to hemolyic anemia
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30
Q

Endothelial damage and inappropriate platelet aggregation lead to significant morbodity in HUS. How?
3

A

Microangiopathic hemolytic anemia

Acute kidney injury and renal failure

Thrombocytopenia

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31
Q

Signs and Symptoms of HUS

8

A
  1. Recent or current bloody diarrhea
  2. Abdominal pain
  3. Decreased urine output
  4. Hematuria
  5. Renal failure
  6. Hypertension
  7. Neurologic changes
  8. Edema
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32
Q

Treatment of HUS?

4

A

Generally only supportive measurements:

  1. Transfuse RBCs and platelets if needed
  2. Dialysis if symptomatic uremia
  3. Nutritional and electrolyte support
  4. If thought to be secondary to an autoimmune process may consider plasma exchange
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33
Q

Who is HUS more common is?

A

children

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34
Q

MAjor things HUS is caused by?

4

A

E. Coli
Strep Pneumo
Shigella
meds

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35
Q

What are the four major symtpoms associated with HSP (Henoch-Schönelin purpura)?
4

A

Palpable purpura
Arthritis/arthralgias
Abdominal pain
Renal disease

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36
Q

What is HSP?

A

IgA vasculitis

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37
Q

In who do most cases of HSP occur in?

A

3-15 yo

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38
Q

What can HSP be triggered by?

A

streptococcal upper respiratory infection

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39
Q

What should we not see in patients with HSP that would disclude other diseases

A

thrombocytopenia or coagulopathy

no anemia or bleeding

40
Q

How do we treat HSP?

2

A

Treatment is supportive with NSAIDs or glucocorticoids

Renal failure from IgA deposition may not be evident for 6 months so follow up is necessary

41
Q

What lab tests would we do for HSP?

4

A
  1. biopsy of the skin lesions,
  2. CBC,
  3. CMP,
  4. urinalysis (may show hematuria, +/- rbc casts, +/- proteinuria)
42
Q

What are the three bleeding disorders we talked about?

A

Hemophilia
VonWillebrand’s disease
Disseminated intravascular coagulation

43
Q

What is hemophilia A caused by?

What is hemophilia B caused by?

What is hemophilia C caused by?

WHat is the most common and the rarest?

A

Factor VIII deficiency

Factor IX deficiency

Factor XI deficiency

A is the most common 80%
C is the rarest

44
Q

What kind of bleeding is hemophilia caused by?

What are the most common bleeding sites and what is the most dangerous that we are at risk for?
5

A

Characteristic of this disease is spontaneous hemarthrosis (bleeding into the joint. not trauma induced)

At risk for spontaneous intracerebral hemorrhage
Most common bleeding sites:

Joints: knees, ankles, elbows
Skin, muscle
GU, GI

45
Q

What is a major complication of hemophilia and what can it lead to?

A

Hemophillic arthropathy

Development of antibodies to the clotting factor concentrate that we treat with

46
Q

What is the treatment for hemophilia?

A

lifelong replacement of deficient clotting factor.

-cyroprecipitate

47
Q

How often do we replace the clotting factor and how?

A

usually 3 times per week

IV bolus for home administration

48
Q

Why would we give an extra dose of clotting factor to hemophilia pts?

A

Extra dose given if needed due to trauma or suspicion of joint bleeding

49
Q

What should we avoid with hemophilia pts?

A

avoid aspirin

50
Q

What other medications could we give them?

A

DDVAP

51
Q

What lab diagnostics would we use to diagnose hemophilia?

2 things that are positive to diagnose

4 that should be normal and rule out other disease

A
  1. PTT is prolonged (PTT associated with factor 8)
  2. Diagnosis is confirmed by decreased levels of Factor VIII, IX or XI
  3. PT (extrinsic pathway), Bleeding time,
    Platelets,
    vonWillebrand factor are normal
52
Q

Do hemophiliacs

a) bleed longer
b) bleed more quickly
c) have a shortened life span

A

a. yes
b. no
c. only if they dont have access to factor replacement therapy

53
Q

What are the two main funcitons of von Willebrand factor?

A
  1. Binds platelets to form the initial platelet plug

2. Binds with Factor VIII to prolong it’s half life

54
Q

HOw is VWD inherited?

Which type is the most common?

A

Autosomal dominant affecting both sexes equally
most common inherited bleeding disorder

Type1

55
Q

Pathology of Type 1 VWD?

A

Quantitative abnormality of vWF (molecule is normal but they just dont have enough)

56
Q

Pathology of type 2 VWD?

What does it clinically resemble?

A
  1. Qualitative abnormality
  2. Decreased binding to factor VIII and platelets (doesnt bind the way it should)

Clinically resembles hemophilia A

57
Q

Pathology of Type 3 VWD?

A

Rare

Undetectable levels of vWF and severe bleeding in infancy and childhood

58
Q

Symtpoms of VWD?

3

A
  1. Easy bruising,
  2. skin bleeding,
  3. prolonged bleeding from mucosal surfaces
59
Q

Personal and family history questions for the diagnosis of WVD?
4

A
  1. Nosebleeds > 10 min in childhood
  2. Lifelong easy bruising (associated hematoma)
  3. Bleeding following dental extractions or other surgery
  4. Heavy menstrual bleeding or post partum
60
Q

What lab tests would we do to diagnose VWD?

Three would test positive for VWD

Two tests that should be normal for VWD

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

61
Q

How would we treat Type 1 VWD for:

Minor Bleeding
Major bleeding

A

DDAVP

DDAVP, vWF concentrate

62
Q

HOw would we treat Type 2 VWD for:
Minor bleeding
Major bleeding

A

DDAVP, vWF concentrate

vWF concentrate

63
Q

HOw would you treat type 3 VWD for:
Minor bleeding
Major bleeding

A

vWF concentrate

vWF concentrate

64
Q

What treatment would we avoid?

A

aspirin or other NSAIDs

65
Q

What causes DIC?

WHat does this set off?
4

A

Massive release of tissue factor (sepsis or trauma)

  1. Tissue factor then sets the coagulation system in place
  2. Coagulation occurs
  3. Clotting factors and inhibitors are consumed
  4. Clots further trap circulating platelets leading to ischemia (from the clots)
66
Q

Widespread clotting occurs in DIC as mentioned above. What is occurring simultaneously?

A

excess thrombin activates plasmin resulting in fibrinolysis.

67
Q

What does fibrinolysis caused by the excess thrombin (breakdown of clots) result in?

Besides fibrinolysis, what does plasmin also activate?

A

fibrin degradation products which have further anticoagulant properties

Plasmin also activates the complement and kinin systems = shock
-inflammation (kinin) = dilation = shock

68
Q

Causes of DIC?

5

A 
Cancer
Obstetric complications
Sepsis (infection)
Massive tissue injury
-Trauma
-Burns
-hyperthermia
Snake bite
69
Q

Clinical manifestations of DIC?

6

A 
Thrombosis and hemorrhage
Petechia, purpura, 
gangrene
Renal failure, 
liver failure

In cancer patients it can be of slow onset

70
Q

What labs would we do to diagnose DIC?

5

A
  1. Thrombocytopenia
  2. Prolongation of PT and PTT (dont have enough clotting factors left)
  3. Low fibrinogen (has been converted to fibrin for the clots)
  4. Increased levels of fibrinogen degradation products (d-Dimer)-lab test
  5. Schistocytes (helmet cells)- torn in half
71
Q

What is treatment targeted at in DIC?

A

targeted at the underlying cause and is otherwise supportive.

72
Q

When would we use anticoagulants in treatment of DIC?

Why only in this case?

What about if the PT/INR is significantly elevated in DIC?

A

Anticoagulants only to prevent imminent death
-clot in the lungs for example. only if it outweighs the risk

going to shift. will now be clotting more and higher risk for losing extremities

Fresh frozen plasma or cryoprecipitate to keep fibrinogen > 100 mg/dL

73
Q

What are the factors that cause hypercoaguable states (clotting disorders)?

What are some examples of things that cause endothelial damage that could cause clotting?4

A
  1. Endothelial damage and inflammation
  2. Elevated platelet levels

Atherosclerosis
Diabetes mellitus
Tobacco use
Cancers

74
Q

Conditions that accelerate the activity of the clotting system?
9

A 
Pregnancy
Oral contraceptives
Postsurgical state
Malignancy
Hereditary clotting disorders
Stasis/Immobility
Low cardiac output (CHF)
Obesity
Sleep apnea
75
Q

What does Protein C do?

What will a deficiency in protein C result in?

A

inactivates Factors V and VIII thereby inhibiting coagulation

leads to prolonged action of Factors V and VIII leading to excessive clotting

76
Q

Signs and symptoms of protein C deficiency?

4

A

Thrombosis
Deep vein thrombosis
Pulmonary embolism
Thrombophlebitis

77
Q

What labs would we do to daignose protein C deficiency?

5

A
  1. Protein C (low)
  2. PTT, PT (see if extrinsic and intrinsic pathways work-they should)
  3. Thrombin time (fast)
  4. Bleeding time (slow)
  5. Medical and family history may be revealing for a history of thromboembolism
78
Q

How would we treat Protein C?

2

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

Dont treat unless they have clot formation
No prophylactic treatment

79
Q

What is the funciton of protein S?

What would a deficiency is protein C cause?

A

Protein S is 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

80
Q

Symptoms, diagnosis and treatment for protein S deficiency?

A

same as protein C

81
Q

How is antithrombin deficiency inherited?

A

Usually autosomal dominant

82
Q

Cinical manifestations?

3

A

Recurrent venous thrombosis, pulmonary embolism and repetitive intrauterine fetal death

83
Q

When is the peak age of onset for antithrombin deficiency?

A

15-35 yo

84
Q

What is antithrombins function?

A

regulate clotting

-Major inhibitor of thrombin and other clotting Factors including X and IX

85
Q

What does antithrombin deficiency lead to?

A

increased tendency to form clots.

-no inhibtion of clotting factors so they are in the blood stream longer = increasing risk of thrmobus formation

86
Q

Lab tests for diagnosing antithrombin deficiency?

What will indicate the disease and what will be normal?

A
  1. Antithrombin-heparin cofactor assay is the best screening test for AT deficiency
    - -Measures functional AT activity

If patients are on anticoagulants or are in the midst of a thrombotic crisis the test results can be skewed (same for Protein C & S)

Standard coagulation tests should be normal (PT, PTT)

87
Q

How do we treat antithrombin deficiency?

A

same as protein C and S disorders

88
Q

Whats the most common genetic disorder to cause DVT?

A

Factor V Leiden

89
Q

What does a lack of factor V Leiden result in?

A

Blood has an increased tendency to clot and is most likely to occur in the veins

Lack of Factor V Leiden decreases the anticoagulant activity of the activated protein C

90
Q

Factor V Leiden Disease is asymptomatic until a clot forms resulting in the following disorders?
3

A

Thrombophlebitis
Deep vein thrombosis
Pulmonary embolism

91
Q

Treatment of Factor V Leiden Disease?

A

Chronic anticoagulation if presenting with blood clots

If no history of thrombosis then only use anticoagulants if at risk for clots due to hospitalization or surgery

92
Q

What is Antiphospholipid antibody?

A

Autoimmune hypercoagulable state caused by antiphospholipid antibodies

Antibodies lead to arterial and venous clot formation

Could cause end organ damage

93
Q

What are pregnancy complications of Antiphospholipid antibody?
4

A

miscarriage,
stillbirth,
preterm delivery,
severe eclampsia

94
Q

Examples of Antiphospholipid antibodies?
3

What can these these antibodies cause? 3

A

Lupus anticoagulant

Anti-cardiolipin antibody

Anti-beta2-glycoprotein-I

DVT, stroke, miscarriage

95
Q

Primary or secondary causes of antiphopholipid antibody syndrome?
2

A

Primary
No other related disease to cause abnormal antibodies

Secondary
Other autoimmune disorders such as Lupus

96
Q

How do we diagnose antiphopholipid antibody syndrome?

4

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

97
Q

How do we treat APS?

2

A

chronic anticoagulation and antiplatelet therapy.

Aspirin
Warfarin or other oral long term anticoagulants

Hematology (18 decks)

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