Platelet and Vascular Disorders Flashcards
History and Physical
Manifestations vary with disorder
Acquired vs Inherited
Medications
Type of bleeding
- Mucocutaneous
- Hematoma
- Delayed vs sudden onset
Petechiae
Small red-purple spots
Blood leakage through endothelium
Ecchymosis
Bruising
Larger red-purple spots
Blood escaping into tissue
Purpura
Descriptive term meaning purple
Mucosal Bleeding
Epistaxis (nose bleed)
Gingival
Menorrhagia (heavy menstrual bleeding)
Vascular Disorders
Difficult to diagnose
Often diagnosis of exclusion (ruled out everything else)
Coagulation and platelet testing usually normal (PT, APTT, TT, etc)
Senile Purpura Causes
Acquired
Degeneration of supportive collagen
Senile Purpura Clinical Presentation
Benign
Older adults
Ecchymoses appear spontaneously and with slight pressure
Senile Purpura Laboratory Testing
Normal Testing Results
Senile Purpura Therapy
None
Hereditary Hemorrhagic Telangectasia Cause
(Osler Weber Rendu Disease)
AD Inheritance
Lesions of dilated capillaries with abnormal connective tissue (fragile capillaries)
Hereditary Hemorrhagic Telangectasia Clinical Presentation
Bleeding from lesions
- Mucosal bleeds such as lips, tongue, GI tract, nose bleeds
Hereditary Hemorrhagic Telangectasia Laboratory Testing
Normal Testing Results
Hereditary Hemorrhagic Telangectasia Therapy
Supportive
Ehlers-Danlos Syndrome (Rubber Man) Causes
AD Inheritance
Decreased and abnormal synthesis of collagen
Ehlers-Danlos Syndrome Clinical Presentation
Bleeding due to fragile vessels
Hyperextendable skin, hypermobile joints
Ehlers-Danlos Syndrome Laboratory Testing
Abnormal capillary fragility
Possible abnormal BT
Ehlers-Danlos Syndrome Therapy
Supportive
Platelet Disorders Symptoms
Petechiae
Epistaxis
Mucous membrane bleeding
Easy bruising
Platelet Disorders Classification
Quantitative Decrease in Platelets
Quantitative Increase in Platelets
Qualitative Disorders of Platelets
Quantitative Decrease in Platelets
Hypoplasia of bone marrow
Fanconi’s anemia
Medications
Infection
Radiation
Aplastic Anemia
Myelophthisic Anemia
Quantitative Increase in Platelets
Ineffective Production
Abnormal platelets (decreased survival)
May Hegglin Anomaly
Wiskott-Aldrich Syndrome
Alcohol-related
Quantitative Decrease Platelets Reason
Increased Destruction
- Idiopathic Thrombocytopenic Purpura (ITP)
ITP
Antibody to platelets
Platelets destroyed in spleen
Acute ITP
2-6 yr old
Rapid onset
<20,000/cumm
Treat with steroids
Chronic ITP
20-40 yr old
30,000 - 100,000/cumm
Treat with steroids, splenectomy
Quantitative Decrease in Platelets Reason
Increased destruction
DIC
Thrombotic Thrombocytopenic Purpura
Drug-induced platelet antibodies
Hemolytic Uremic Syndrome
Splenomegaly
Massive Transfusion
Heart Bypass
Reactive Thrombocytosis
Thrombocythemia
DIC
Non-immune consumption of platelets
Thrombotic Thrombocytopenic Purpura
Idiopathic, non-immune
Associated with pregnancy, oral contraceptives, SLE
CNS damage and Sx
Hemolytic Uremic Syndrome (HUS)
Increased BUN = coating of platelets
BT - prolonged
Abnormal aggregation with collagen
Abnormal secondary aggregation with ADP & epinephrine
Splenomegaly
Abnormal distribution
Increased sequestration in the spleen of platelets
Massive Transfusion
10-30 units - dilutional thrombocytopenia
Overly diluted platelets
Heart Bypass
Mechanical damage to platelets
Reactive Thrombocytosis
Secondary to another condition
<1,000,000/cumm
Transient
BT - normal
Platelet function test - normal
Bleeding infrequent
Thrombosis infrequent
Thrombocythemia
Malignant proliferation of megakaryocytes
May be >1,000,000/cumm
Associated with MPD
BT - abnormal
Platelet function test - abnormal
Bleeding - frequent
Thrombosis - frequent
Qualitative Platelet Disorders
Bernard Soulier Sundrome
Glanzman’s Thrombasthenia
Storage Pool Disease
Thromboxane A2 Synthesis
Gray Platelet Syndrome
Uremia
Bernard Soulier Syndrome Cause
AR Inheritance
Lack of GPIb/IX receptor on platelet
Decreased interaction with VWF
Prevents adhesion to collagen
Bernard Soulier Syndrome Clinical Presentation
Mucous membrane bleeding
Many large platelets on PBS
Bernard Soulier Syndrome Laboratory Testing
BT -prolonged
Platelet count - normal to decreased
Platelet aggregation - normal to all except ristocetin
Cannot correct with platelet aggregation with NP
Bernard Soulier Syndrome Platelet Aggregation Pattern
Glanzman’s Thrombasthenia Cause
AR Inheritance
Deficiency of thrombasthenin (contractile protein)
Decrease in GPIIb-IIIa complex
Unable to bind fibrinogen
Glanzman’s Thrombasthenia Clinical Presentation
Moderate bleeding
Glanzman’s Thrombasthenia Laboratory Testing
BT - prolonged
Clot Retraction - Absent
Platelet aggregation - Abnormal to all but ristocetin
Glanzman’s Thrombasthenia Platelet Aggregation Pattern
Storage Pool Disease Cause
AD Inheritance
Deficiency of dense grandules
Decrease release of ADP
Storage Pool Disease Clinical Signs
Mucous membrane bleeding
Storage Pool Disease Laboratory Testing
BT - usually prolonged
Platelet aggregation
- No secondary aggregation with ADP/epinephrine
- No aggregation with collagen
Storage Pool Disease Aggregation Pattern
Aspirin
Uremia
Thromboxane A2 Synthesis Cause
Deficiency of cyclo-oxygenase
Aspirin Ingestion
Thromboxane A2 Synthesis Clinical Signs
Mucous membrane bleeding
Thromboxane A2 Synthesis Laboratory Testing
BT - usually prolonged
Platelet aggregation
- No secondary aggregation with ADP/epinephrine
- No aggregation with collagen
Thomboxane A2 Synthesis Platelet Aggregation Pattern
Gray Platelet Syndrome Cause
Deficiency of alpha granules
Gray Platelet Syndrome Clinical Signs
Mild bleeding - if any
Gray Platelet Syndrome Laboratory Testing
BT - normal
Platelet Count - normal
Clot Retraction - normal
Platelet Aggregation Studies - normal
Platelets appear agranular on PBS
Uremia Cause
Underlying cause of uremia
Uremia Clinical Signs
Mucous membrane bleeding - may be severe
Uremia Laboratory Testing
BT - prolonged
Platelet Count - normal to abnormal
Platelet Aggregation
- decreased with collagen
- decreased secondary aggregation with ADP & epinephrine
VWF Platelet Aggregation Pattern
Myeloproliferative disease Platelet Aggregation Pattern
Normal Platelet Aggregation Pattern