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Heme/Onco > Platelet Disorders > Flashcards

Platelet Disorders Flashcards

1
Q

Thrombocytopenia

Etiology

A
  1. Decreased production (bone marrow disease; nutrition deficiency)
  2. Increased destruction (Medication, alcohol, autoimmune disease, DIC)
  3. Sequestration by spleen
    * First needs to r/o pseudothrombocytopenia (looking for platelet clots)
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2
Q

Thrombocytosis

Etiology

A

Reactive to: infection, post-surgery, splenectomy, malignancy, acute blood loss;
Myeloproliferative disorders

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3
Q

Pseudothrombocytopenia

Etiology

A
  1. Incomplete mixing of blood collection tubes;

2. EDTA dependent agglutinins

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4
Q

Pseudothrombocytopenia

How to identify?

A

Check peripheral blood smear to find platelet clots

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5
Q

What is the lifespan of plt?

A

10 days

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6
Q

Thrombocytopenia

Causes of plt underproduction

A

Marrow failure
Marrow invasion
Marrow injury
Congenital

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7
Q

Thrombocytopenia
Causes of plt underproduction
Marrow failure

A

Aplastic anemia
Myelodysplasia
Vitamin B12/folate deficiency
Fanconi anemia

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8
Q

Thrombocytopenia
Causes of plt underproduction
Marrow invasion

A

Leukemia
Tumors
Granulomatous disease (sarcoidosis)
Fibrosis (primary myelofibrosis)

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9
Q

Thrombocytopenia
Causes of plt underproduction
Marrow injury

A

Drugs (especially alcohol, chemo)
Radiation
Infections (HCV, HIV)

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10
Q

Thrombocytopenia
Causes of plt underproduction
Congenital

A 
Wiskott-Aldrich syndrome
Thrombocytopenia absent radius (TAR) syndrome
May-Hegglin anomaly
Gray platelet syndrome
Bernard-Soulier syndrome
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11
Q

Thrombocytopenia
Platelet sequestration
Will thrombopoietin synthesis increase in the setting of splenic sequestration?

A

No.

Even it is sequested, thrombopoietin is still negatively controlled by plt mass.

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12
Q

Thrombocytopenia
Non-immune-mediated thrombocytopenia
Pathophysiology

A

Microangiopathic

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13
Q

Thrombocytopenia
Non-immune-mediated thrombocytopenia
3 categories

A

DIC
TTP
HUS

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14
Q

Thrombocytopenia
Non-immune-mediated thrombocytopenia
TTP-pathophysiology?

A

Abnormal activation of plt and endothelial cells.

Deficiency in the protease ADAMTS-13

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15
Q

Thrombocytopenia
Non-immune-mediated thrombocytopenia
TTP
What is the causes of deficiency of protease ADAMTS-13

A

Congential;

Produced by an autoantibody

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16
Q

Thrombocytopenia
Non-immune-mediated thrombocytopenia
TTP
What is the consequence of protease ADAMTS-13 deficiency?

A

Accumulation of clumps of ultra-large-molecular-weight vWF multimers–>microvascular occusion–>thrombocytopnenia

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17
Q

Thrombocytopenia
Non-immune-mediated thrombocytopenia
TTP
What are the triggers of TTP?

A

Medication

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18
Q

Thrombocytopenia
Non-immune-mediated thrombocytopenia
TTP
What medications can trigger TTP?

A

Quinine
Ticlopidine
Cyclosporine
Gemcitabine

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19
Q

Thrombocytopenia
Non-immune-mediated thrombocytopenia
TTP
What are the lab findings?

A 
Thrombocytopenia
Schistocytes on peripheral blood smear
Elevated LDH and bilirubin
Low haptoglobin
Low RBC
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20
Q

Thrombocytopenia
Non-immune-mediated thrombocytopenia
TTP
Sx:

A 
Easy bruising;
Fever
Kidney disease
Neurologica changes
Abdominal pain
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21
Q

Thrombocytopenia
Non-immune-mediated thrombocytopenia
TTP
Treatment?

A

Plasma exchange

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22
Q

Thrombocytopenia
Non-immune-mediated thrombocytopenia
TTP
Can you transfuse plt?

A

NO!

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23
Q

Thrombocytopenia
Non-immune-mediated thrombocytopenia
HUS
What are the 2 types?

A

Typical - induced by diarrhea or infection

Atypical - not related to diarrhea episodes

24
Q

Thrombocytopenia
Non-immune-mediated thrombocytopenia
HUS
Typical HUS- infections?

A

E.Coli O157:H7

Shiga toxin producing bacteria

25
Q

Thrombocytopenia
Non-immune-mediated thrombocytopenia
HUS
Treatment for typical HUS-infection?

A

Plasma exchange

26
Q

Thrombocytopenia
Non-immune-mediated thrombocytopenia
Atypical HUS
Causes

A

Caused by congenital syndrome

Overwhelming complement activation

27
Q

Thrombocytopenia
Non-immune-mediated thrombocytopenia
Atypical HUS treatment?

A

Eculizumab

28
Q

Thrombocytopenia
Non-immune-mediated thrombocytopenia
Atypcial HUS
Mechanism of eculizumab?

A

Monoclonal antibody directed against terminal components of the complement cascade.

29
Q

Thrombocytopenia
Immune-mediated thrombocytopenia
ITP
Will you do antiplatelet antibody test?

A

NO!

Low sensitivity and low specificity

30
Q

Thrombocytopenia
Immune-mediated thrombocytopenia
ITP
What are the causes of ITP

A 
Medications;
CLL
HL
NHL
Viral illness (HIV. HCV)
31
Q

Thrombocytopenia
Immune-mediated thrombocytopenia
ITP
What are the lab findings if ITP?

A

Normal Hgb and WBC
Large plt on peripheral blood smear
No schistocyte of peripheral blood smear
R/o hyper/hypo thyroidism

32
Q

Thrombocytopenia
Immune-mediated thrombocytopenia
ITP
When to treat?

A

If plt<30 or bleeding

33
Q

Thrombocytopenia
Immune-mediated thrombocytopenia
ITP
What to treat?

A

Prednisone 1mg/kg followed by slow taper over the course of weeks

34
Q

Thrombocytopenia
Immune-mediated thrombocytopenia
ITP
What if not responding or relapse after steroids taper?

A

IVIG and anti-D immune globulin

35
Q

Thrombocytopenia
Immune-mediated thrombocytopenia
ITP
When to start Anti-D immune globulin

A

Only in Rh-positive patients

36
Q

Thrombocytopenia
Immune-mediated thrombocytopenia
ITP
What are the 2nd line therapies

A

Rituximab
Splenectomy
Use of thrombopoietin mimetic agents

37
Q

Thrombocytopenia
Immune-mediated thrombocytopenia
ITP
How to treat if plt<10?

A

IVIG+ steroids+ transfusion

38
Q

Thrombocytopenia
Immune-mediated thrombocytopenia
HIT (Heparin-induced thrombocytopenia)
Pathophysiology?

A

Antigen: complex between flatelet factor (PF4) and heparin;
Antibody: activate PF-4 and heparin through Fc receptor on platelet

39
Q

Thrombocytopenia
Immune-mediated thrombocytopenia
HIT
Sx:

A

Plt decrease by 50% 5-10 days after treatment of heparin;

Could also be thrombosis

40
Q

Thrombocytopenia
Immune-mediated thrombocytopenia
HIT
Diagnostic test?

A 
Serologic testing (ELISA): 
Functional assay: serotonin release assay (gold standard)
41
Q

Thrombocytopenia
Immune-mediated thrombocytopenia
HIT
Management

A

Stop Heparin, start argatroban;
Send serologic testing and functional assay;
Baseline doppler ultrasound of all 4 extremities

42
Q

Thrombocytopenia
Immune-mediated thrombocytopenia
HIT
Predicting HIT pretest probability

A 
4Ts
Thrombocytopnenia
Timing of platelet count decrease
Thrombosis other sequelae
Other causes for thrombocytopenia
43
Q

Platelet function test

When to do the test?

A

If abnormal bleeding in those plt count, PT and PTT are normal

44
Q

Platelet dysfunction

What are the congenital platelet defects?

A 
Glanzmann thrombasthenia;
Bernard-Soulier syndrome
Wiskott-Aldrich syndrome
Gray pletelet syndrome
Storage pool disease
45
Q

Platelet dysfunction
Glanzmann thrombasthenia
Mechanism

A

Defect in glycoprotein IIb-IIIa

Severe

46
Q

Platelet dysfunction
Glanzmann thrombasthenia
Treatment

A

Platelets or recombinant factor VIIa

e-aminocaproic acid

47
Q

Platelet dysfunction
Bernard-Soulier syndrome
Mechanism

A

Defect in glycoprotein Ib-IX

Severe

48
Q

Platelet dysfunction
Bernard-Soulier syndrome
Treatment

A

Platelet

e-aminocaproic acid

49
Q

Platelet dysfunction
Wiskott-Aldrich syndrome
Mechanism

A

Triad of eczema, thrombocytopenia and immunodeficiency

Moderate

50
Q

Platelet dysfunction
Wiskott-Aldrich syndrome
Treatment

A

Platelet

e-aminocaproic acid

51
Q

Platelet dysfunction
Gray platelet syndrome
Symptoms

A

May develop myelofibrosis

Moderate

52
Q

Platelet dysfunction
Gray platelet syndrome
Rx

A

Platelet

e-aminocaproic acid

53
Q

Platelet dysfunction
Storage pool disease
Mechanism

A

Defects in the granules in platelets, lack of granular non-metabolic ADP
Moderate to mild

54
Q

Platelet dysfunction
Storage pool disease
Rx

A

Platelet
e-aminocaproic acid
May resonpde to desmopressin

55
Q

Platelet dysfunction

What are the acquired platelet defects?

A 
Uremia;
Liver disease
Myeloproliferative neoplasms;
Post-cardiac bypass
Antiplatelet drugs;
IIb-IIIa inhibitors
Aspirin
Clopidogrel
NSAIDs;
Other drugs and herbs (beta-lactams, vitamin E, ginkgo, turmeric, garlic, Chinese ree fungus)
56
Q

Platelet dysfunction

Are aspirin and clopidogrel induced thrombopenia reversible?

A

No. Irreversible.

Heme/Onco (19 decks)

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