Platelet and Vascular Disorders

Question 1

History and Physical

Answer 1

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

Question 2

Petechiae

Answer 2

Small red-purple spots
Blood leakage through endothelium

Question 3

Ecchymosis

Answer 3

Bruising
Larger red-purple spots
Blood escaping into tissue

Question 4

Purpura

Answer 4

Descriptive term meaning purple

Question 5

Mucosal Bleeding

Answer 5

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

Question 6

Vascular Disorders

Answer 6

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

Question 7

Senile Purpura Causes

Answer 7

Acquired
Degeneration of supportive collagen

Question 8

Senile Purpura Clinical Presentation

Answer 8

Benign
Older adults
Ecchymoses appear spontaneously and with slight pressure

Question 9

Senile Purpura Laboratory Testing

Answer 9

Normal Testing Results

Question 10

Senile Purpura Therapy

Answer 10

None

Question 11

Hereditary Hemorrhagic Telangectasia Cause
(Osler Weber Rendu Disease)

Answer 11

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

Question 12

Hereditary Hemorrhagic Telangectasia Clinical Presentation

Answer 12

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

Question 13

Hereditary Hemorrhagic Telangectasia Laboratory Testing

Answer 13

Normal Testing Results

Question 14

Hereditary Hemorrhagic Telangectasia Therapy

Answer 14

Supportive

Question 15

Ehlers-Danlos Syndrome (Rubber Man) Causes

Answer 15

AD Inheritance
Decreased and abnormal synthesis of collagen

Question 16

Ehlers-Danlos Syndrome Clinical Presentation

Answer 16

Bleeding due to fragile vessels
Hyperextendable skin, hypermobile joints

Question 17

Ehlers-Danlos Syndrome Laboratory Testing

Answer 17

Abnormal capillary fragility
Possible abnormal BT

Question 18

Ehlers-Danlos Syndrome Therapy

Answer 18

Supportive

Question 19

Platelet Disorders Symptoms

Answer 19

Petechiae
Epistaxis
Mucous membrane bleeding
Easy bruising

Question 20

Platelet Disorders Classification

Answer 20

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

Question 21

Quantitative Decrease in Platelets

Answer 21

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

Question 22

Quantitative Increase in Platelets

Answer 22

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

Question 23

Quantitative Decrease Platelets Reason

Answer 23

Increased Destruction
- Idiopathic Thrombocytopenic Purpura (ITP)

Question 24

ITP

Answer 24

Antibody to platelets
Platelets destroyed in spleen

Question 25

Acute ITP

Answer 25

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

Question 26

Chronic ITP

Answer 26

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

Question 27

Quantitative Decrease in Platelets Reason

Answer 27

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

Question 28

DIC

Answer 28

Non-immune consumption of platelets

Question 29

Thrombotic Thrombocytopenic Purpura

Answer 29

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

Question 30

Hemolytic Uremic Syndrome (HUS)

Answer 30

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

Question 31

Splenomegaly

Answer 31

Abnormal distribution Increased sequestration in the spleen of platelets

Question 32

Massive Transfusion

Answer 32

10-30 units - dilutional thrombocytopenia Overly diluted platelets

Question 33

Heart Bypass

Answer 33

Mechanical damage to platelets

Question 34

Reactive Thrombocytosis

Answer 34

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

Question 35

Thrombocythemia

Answer 35

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

Question 36

Qualitative Platelet Disorders

Answer 36

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

Question 37

Bernard Soulier Syndrome Cause

Answer 37

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

Question 38

Bernard Soulier Syndrome Clinical Presentation

Answer 38

Mucous membrane bleeding Many large platelets on PBS

Question 39

Bernard Soulier Syndrome Laboratory Testing

Answer 39

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

Question 40

Bernard Soulier Syndrome Platelet Aggregation Pattern

Answer 40

Question 41

Glanzman's Thrombasthenia Cause

Answer 41

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

Question 42

Glanzman's Thrombasthenia Clinical Presentation

Answer 42

Moderate bleeding

Question 43

Glanzman's Thrombasthenia Laboratory Testing

Answer 43

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

Question 44

Glanzman's Thrombasthenia Platelet Aggregation Pattern

Answer 44

Question 45

Storage Pool Disease Cause

Answer 45

AD Inheritance Deficiency of dense grandules Decrease release of ADP

Question 46

Storage Pool Disease Clinical Signs

Answer 46

Mucous membrane bleeding

Question 47

Storage Pool Disease Laboratory Testing

Answer 47

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

Question 48

Storage Pool Disease Aggregation Pattern Aspirin Uremia

Answer 48

Question 49

Thromboxane A2 Synthesis Cause

Answer 49

Deficiency of cyclo-oxygenase Aspirin Ingestion

Question 50

Thromboxane A2 Synthesis Clinical Signs

Answer 50

Mucous membrane bleeding

Question 51

Thromboxane A2 Synthesis Laboratory Testing

Answer 51

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

Question 52

Thomboxane A2 Synthesis Platelet Aggregation Pattern

Answer 52

Question 53

Gray Platelet Syndrome Cause

Answer 53

Deficiency of alpha granules

Question 54

Gray Platelet Syndrome Clinical Signs

Answer 54

Mild bleeding - if any

Question 55

Gray Platelet Syndrome Laboratory Testing

Answer 55

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

Question 56

Uremia Cause

Answer 56

Underlying cause of uremia

Question 57

Uremia Clinical Signs

Answer 57

Mucous membrane bleeding - may be severe

Question 58

Uremia Laboratory Testing

Answer 58

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

Question 59

VWF Platelet Aggregation Pattern

Answer 59

Question 60

Myeloproliferative disease Platelet Aggregation Pattern

Answer 60

Question 61

Normal Platelet Aggregation Pattern

Answer 61