Platelet and Vascular Disorders Flashcards

1
Q

History and Physical

A

Manifestations vary with disorder
Acquired vs Inherited
Medications
Type of bleeding
- Mucocutaneous
- Hematoma
- Delayed vs sudden onset

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2
Q

Petechiae

A

Small red-purple spots
Blood leakage through endothelium

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3
Q

Ecchymosis

A

Bruising
Larger red-purple spots
Blood escaping into tissue

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4
Q

Purpura

A

Descriptive term meaning purple

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5
Q

Mucosal Bleeding

A

Epistaxis (nose bleed)
Gingival
Menorrhagia (heavy menstrual bleeding)

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6
Q

Vascular Disorders

A

Difficult to diagnose
Often diagnosis of exclusion (ruled out everything else)
Coagulation and platelet testing usually normal (PT, APTT, TT, etc)

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7
Q

Senile Purpura Causes

A

Acquired
Degeneration of supportive collagen

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8
Q

Senile Purpura Clinical Presentation

A

Benign
Older adults
Ecchymoses appear spontaneously and with slight pressure

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9
Q

Senile Purpura Laboratory Testing

A

Normal Testing Results

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10
Q

Senile Purpura Therapy

A

None

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11
Q

Hereditary Hemorrhagic Telangectasia Cause
(Osler Weber Rendu Disease)

A

AD Inheritance
Lesions of dilated capillaries with abnormal connective tissue (fragile capillaries)

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12
Q

Hereditary Hemorrhagic Telangectasia Clinical Presentation

A

Bleeding from lesions
- Mucosal bleeds such as lips, tongue, GI tract, nose bleeds

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13
Q

Hereditary Hemorrhagic Telangectasia Laboratory Testing

A

Normal Testing Results

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14
Q

Hereditary Hemorrhagic Telangectasia Therapy

A

Supportive

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15
Q

Ehlers-Danlos Syndrome (Rubber Man) Causes

A

AD Inheritance
Decreased and abnormal synthesis of collagen

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16
Q

Ehlers-Danlos Syndrome Clinical Presentation

A

Bleeding due to fragile vessels
Hyperextendable skin, hypermobile joints

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17
Q

Ehlers-Danlos Syndrome Laboratory Testing

A

Abnormal capillary fragility
Possible abnormal BT

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18
Q

Ehlers-Danlos Syndrome Therapy

A

Supportive

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19
Q

Platelet Disorders Symptoms

A

Petechiae
Epistaxis
Mucous membrane bleeding
Easy bruising

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20
Q

Platelet Disorders Classification

A

Quantitative Decrease in Platelets
Quantitative Increase in Platelets
Qualitative Disorders of Platelets

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21
Q

Quantitative Decrease in Platelets

A

Hypoplasia of bone marrow
Fanconi’s anemia
Medications
Infection
Radiation
Aplastic Anemia
Myelophthisic Anemia

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22
Q

Quantitative Increase in Platelets

A

Ineffective Production
Abnormal platelets (decreased survival)
May Hegglin Anomaly
Wiskott-Aldrich Syndrome
Alcohol-related

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23
Q

Quantitative Decrease Platelets Reason

A

Increased Destruction
- Idiopathic Thrombocytopenic Purpura (ITP)

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24
Q

ITP

A

Antibody to platelets
Platelets destroyed in spleen

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25
Q

Acute ITP

A

2-6 yr old
Rapid onset
<20,000/cumm
Treat with steroids

26
Q

Chronic ITP

A

20-40 yr old
30,000 - 100,000/cumm
Treat with steroids, splenectomy

27
Q

Quantitative Decrease in Platelets Reason

A

Increased destruction
DIC
Thrombotic Thrombocytopenic Purpura
Drug-induced platelet antibodies
Hemolytic Uremic Syndrome
Splenomegaly
Massive Transfusion
Heart Bypass
Reactive Thrombocytosis
Thrombocythemia

28
Q

DIC

A

Non-immune consumption of platelets

29
Q

Thrombotic Thrombocytopenic Purpura

A

Idiopathic, non-immune
Associated with pregnancy, oral contraceptives, SLE
CNS damage and Sx

30
Q

Hemolytic Uremic Syndrome (HUS)

A

Increased BUN = coating of platelets
BT - prolonged
Abnormal aggregation with collagen
Abnormal secondary aggregation with ADP & epinephrine

31
Q

Splenomegaly

A

Abnormal distribution
Increased sequestration in the spleen of platelets

32
Q

Massive Transfusion

A

10-30 units - dilutional thrombocytopenia
Overly diluted platelets

33
Q

Heart Bypass

A

Mechanical damage to platelets

34
Q

Reactive Thrombocytosis

A

Secondary to another condition
<1,000,000/cumm
Transient
BT - normal
Platelet function test - normal
Bleeding infrequent
Thrombosis infrequent

35
Q

Thrombocythemia

A

Malignant proliferation of megakaryocytes
May be >1,000,000/cumm
Associated with MPD
BT - abnormal
Platelet function test - abnormal
Bleeding - frequent
Thrombosis - frequent

36
Q

Qualitative Platelet Disorders

A

Bernard Soulier Sundrome
Glanzman’s Thrombasthenia
Storage Pool Disease
Thromboxane A2 Synthesis
Gray Platelet Syndrome
Uremia

37
Q

Bernard Soulier Syndrome Cause

A

AR Inheritance
Lack of GPIb/IX receptor on platelet
Decreased interaction with VWF
Prevents adhesion to collagen

38
Q

Bernard Soulier Syndrome Clinical Presentation

A

Mucous membrane bleeding
Many large platelets on PBS

39
Q

Bernard Soulier Syndrome Laboratory Testing

A

BT -prolonged
Platelet count - normal to decreased
Platelet aggregation - normal to all except ristocetin
Cannot correct with platelet aggregation with NP

40
Q

Bernard Soulier Syndrome Platelet Aggregation Pattern

41
Q

Glanzman’s Thrombasthenia Cause

A

AR Inheritance
Deficiency of thrombasthenin (contractile protein)
Decrease in GPIIb-IIIa complex
Unable to bind fibrinogen

42
Q

Glanzman’s Thrombasthenia Clinical Presentation

A

Moderate bleeding

43
Q

Glanzman’s Thrombasthenia Laboratory Testing

A

BT - prolonged
Clot Retraction - Absent
Platelet aggregation - Abnormal to all but ristocetin

44
Q

Glanzman’s Thrombasthenia Platelet Aggregation Pattern

45
Q

Storage Pool Disease Cause

A

AD Inheritance
Deficiency of dense grandules
Decrease release of ADP

46
Q

Storage Pool Disease Clinical Signs

A

Mucous membrane bleeding

47
Q

Storage Pool Disease Laboratory Testing

A

BT - usually prolonged
Platelet aggregation
- No secondary aggregation with ADP/epinephrine
- No aggregation with collagen

48
Q

Storage Pool Disease Aggregation Pattern
Aspirin
Uremia

49
Q

Thromboxane A2 Synthesis Cause

A

Deficiency of cyclo-oxygenase
Aspirin Ingestion

50
Q

Thromboxane A2 Synthesis Clinical Signs

A

Mucous membrane bleeding

51
Q

Thromboxane A2 Synthesis Laboratory Testing

A

BT - usually prolonged
Platelet aggregation
- No secondary aggregation with ADP/epinephrine
- No aggregation with collagen

52
Q

Thomboxane A2 Synthesis Platelet Aggregation Pattern

53
Q

Gray Platelet Syndrome Cause

A

Deficiency of alpha granules

54
Q

Gray Platelet Syndrome Clinical Signs

A

Mild bleeding - if any

55
Q

Gray Platelet Syndrome Laboratory Testing

A

BT - normal
Platelet Count - normal
Clot Retraction - normal
Platelet Aggregation Studies - normal
Platelets appear agranular on PBS

56
Q

Uremia Cause

A

Underlying cause of uremia

57
Q

Uremia Clinical Signs

A

Mucous membrane bleeding - may be severe

58
Q

Uremia Laboratory Testing

A

BT - prolonged
Platelet Count - normal to abnormal
Platelet Aggregation
- decreased with collagen
- decreased secondary aggregation with ADP & epinephrine

59
Q

VWF Platelet Aggregation Pattern

60
Q

Myeloproliferative disease Platelet Aggregation Pattern

61
Q

Normal Platelet Aggregation Pattern