Chapter 140 Disorders of Platelets and Vessel Wall Flashcards

1
Q

Cutoff for thrombocytopenia?

A

Platelet <150,000/uL

Normal 150,000-450,000

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2
Q

Major regulator of platelet production

A

Thrombopoietin (TPO)

Synthesized in the liver
Increased in inflammation, by IL-6

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3
Q

Life span of platelets?

A

7-10 days

1/3 of platelets reside in the spleen

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4
Q

Next step in the algorithm if thrombocytopenia is accompanied by abnormal Hgb and WBC?

A

Bone marrow examination

Exception: Isolated thrombocytopenia in patients >60 years, also do bone marrow studies to rule out myelodysplasia

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5
Q

Pinpoint non blanching hemorrhages that are usually the sign of decreased platelet NUMBER and not platelet dysfunction?

Where are these hemorrhages usually seen?

A

Petechiae

Appear in areas of increased venous pressure - ankles and feet

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6
Q

Common bacterial or viral infections that cause infection induced thrombocytopenia?

A

Gram negative bacteria
Infectious mononucleosis
HIV

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7
Q

Mainstay work up for diagnosis of IIT?

A

Bone marrow examination

Blood cultures

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8
Q

When does thrombocytopenia occur when exposed to platelet IIB/IIIA inhibitory drugs?
Examples of these drugs?

A

Within 24 hours of initial exposure

Abciximab

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9
Q

When does thrombocytopenia occur in drug induced thrombocytopenia?

A

After initial exposure, median of 21 days
Or upon reexposure

Resolves 7-10 days after drug withdrawal

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10
Q

Two main differences of HIT from other drug induces thrombocytopenia?

A

Thrombocytopenia not severe

HIT is not associated with bleeding, it actually markedly increases risk for thrombosis

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11
Q

Pathogenesis of HIT?

A

Development of antibodies to heparin/PF4 complex

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12
Q

HIT is more common after exposure to: UFH or LMWH?

A

More common in UFH exposure

After 5-14 days of exposure

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13
Q

Diagnostic algorithm for HIT (4T’s)

A

Thrombocytopenia
Timing of platelet count drop
Thrombosis
Localized skin reactions

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14
Q

How long do heparin/PF4 complexes stay in circulation before disappearing?

A

~100 days following a prior exposure

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15
Q

Rare type of HIT that occurs after all heparin has been stopped, beyond 14 days since last exposure

A

Delayed onset HIT

Heparin/PF4 antibody testing is markedly positive

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16
Q

Diagnostic test for HIT that has high specificity but low sensitivity, may be falsely high in patients who have undergone cardiopulmonary bypass surgery?

A

IgG specific ELISA to PF4 complex

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17
Q

Diagnostic test for HIT which measures the ability of the patient’s serum to activate platelets in the presence of heparin?

A

Serotonin release assay (a platelet activation assay)

Lower sensitivity, higher specificity than ELISA

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18
Q

Most common complication of HIT even after discontinuation of heparin?

A

Thrombosis, venous and arterial

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19
Q

What imaging modality is recommended to evaluate patients suspected of having HIT?

A

Duplex scan of the lower extremities to work up for thrombosis

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20
Q

Which direct thrombin inhibitors (DTI) can be used as alternative for heparin in HITT?

A

Argatroban
Lepirudin

Others not approved by FDA:
Fondaparinux
Bivalirudin

21
Q

Alternative treatment for HIT which is a mixture of glycosaminoglycans with anti Xa activity?

A

Danaparoid

22
Q

If anticoagulation is needed due to high risk of thrombosis in HIT, what drug can be given in place of heparin?

A

Warfarin, usually for 3-6 months

Introduction of warfarin alone may precipitate thrombosis due to clotting activation and reduced protein C and S
Should be overlapped with DTI to lessen prothrombotic state

23
Q

What underlying disorders can cause secondary ITP?

A

Autoimmune disorders such as SLE

Infections such as HIV and Hepatitis C

24
Q

Expected results of platelet count and blood smear in ITP?

A

Very low platelet count

Normal peripheral blood smear

25
What PE findings herald life threatening bleeding in ITP?
Wet purpura (blood blisters in the mouth) and Retinal hemorrhages
26
When is lab testing recommended for ITP?
To evaluate for secondary causes: tests for HIV Hep C H pylori Protein electrophoresis for SLE HIV
27
What test should be performed in ITP with accompanying anemia to rule out combined autoimmune HA (Evans Syndrome)?
Direct Coombs test
28
Outpatient treatment of ITP include:
Prednisone is 1mg/kg - first line treatment, 90% will relapse, remission in 1-3 weeks IVIgG > Anti RhO - given at the 1-2g/kg over 1-5 days, used for acute bleeding risk
29
Which set of ITP patients warrant admission?
Severe thrombocytopenia <5,000 | Signs of impending bleeding such as retinal hemorrhage or large oral mucosal hemorrhage
30
What anti CD20 B cell antibody has shown efficacy in treatment of refractory ITP?
Rituximab
31
What type of vaccination is recommended prior to splenectomy?
Vaccination against encapsulated organisms (pneumococcus, meningococcus, haemophilus)
32
Pentad of findings in TTP
1. Microangiopathic hemolytic anemia 2. Thrombocytopenia 3. Renal failure 4. Neurologic findings 5. Fever
33
Pathogenesis of TTP is due to deficiency of:
ADAMTS13 (deficiency or antibodies against this metalloproteinase) - cleaves vWF Show symptoms of TTP if assay activity level is <10%
34
Expected finding in peripheral smear of TTP?
Presence of schistocyte | Also with polychromasia
35
Mainstay treatment for TTP?
Plasma exchange using FFP Continued until platelet count is normal and hemolysis is resolved for 2 days
36
Triad of symptoms of HUS?
1. Acute renal failure 2. Microangiopathic hemolytic anemia 3. Thrombocytopenia
37
Most frequent etiologic serotype of HUS?
E coli O157:H7
38
Presenting symptom of HUS in children?
Episodes of diarrhea usually hemorrhagic in nature
39
Treatment of HUS?
Supportive, symptom relief of diarrhea May also do plasma exchange Hemodialysis for AKI if needed Treatment from atypical HUS - Eculizumab
40
Most common type of VWD?
Type 1 Disease - decrease in VWF protein/function and Factor VIII
41
Common manifestation of VWD in childhood and in young women?
Children - excessive bruising and epistaxis Young women - menorrhagia Can also present as excessive post op bleeding after dental extraction
42
Mainstay of treatment for patients with VWD?
DDVAP (Desmopressin) Given IV or via intranasal spray results in release of VWD and Factor VIII from endothelial stores
43
Major side effect of DDVAP?
Hyponatremia Due to decreased free water clearance Should advise fluid restriction
44
Drug that can be given to patients with VWD as prophylaxis for dental procedures, tonsillectomy, prostate procedures? But should be used in caution in upper urinary tract bleeding (may cause obstruction)
Tranexamic acid | Antifibrinolytic drugs
45
Screening test for hemophilia?
Prolonged aPTT | Playelets and other bleeding parameters are usually normal
46
Transfusion of what blood product is CONTRAINDICATED for TTP/HUS (unless with active bleeding)?
Transfusion of platelet concentrate
47
Erythromelalgia is characterized as
Erythema, burning, pain in the extremities
48
Thrombosis of what vessel should warrant suspicion of PV
Hepatic vein thrombosis
49
To distinguish PV vs true erythrocytosis
PV - expansion of plasma volume | True erythrocytosis - plasma volume contraction