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62 Cards in this Set
- Front
- Back
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How may vascular abnormalities that cause bleeding appear diagnostically?
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May appear like platelet disorders
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Where are the main problems found with vascular abnormalities that cause bleeding?
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Abnormality in vessels
Abnormality in perivascular connective tissue |
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What kind of bleeding condition is hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu)? (Inherited or acquired)
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Inherited disorder of microvasculature on mucosal surfaces
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How is hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu) inherited?
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Autosomal dominant
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What is the molecular problem that causes hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu)?
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Mutation in endothelial protein, endoglin
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What type of vasculature is affected in hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu) and how is it affected?
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Dilated microvasculature on mucosal surfaces (lips, GI, brain)
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How is hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu) treated?
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Estrogens
Laser embolization |
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What kind of bleeding condition is Ehlers-Danlos syndrome? (Inherited or acquired)
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Inherited disorder of perivascular connective tissue
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What is the pathophysiological basis behind Ehlers-Danlos syndrome?
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Abnormal platelet - connective tissue interactions
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What are the clinical presentations of Ehlers-Danlos syndrome?
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Bruising
Poor skin quality Poor healing Hyperextensibility |
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What kind of bleeding disorder is senile purpura? (Inherited or acquired)
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Acquired disorder of vasculature
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What is the pathophysiological cause of senile purpura?
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Collagen atrophy
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What kind of bleeding disorder is scurvy? (Inherited or acquired)
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Acquired disorder of vasculature
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What causes scurvy?
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Vitamin C deficiency
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What is the pathophysiological cause behind the symptoms of scurvy?
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Collagen defects (from vitamin C deficiency) cause small hemorrhages around hair follicles
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How do steroids affect vasculature?
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Weaken perivascular connective tissue
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What kind of bleeding disorder is Henoch-Schonlein purpura? (Inherited or acquired)
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Acquired disorder of vasculature
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What are the symptoms of Henoch-Schonlein purpura and what is the pathophysiological cause of these symptoms?
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Vasculitis caused by IgA from infection
- Buttocks - Extensor surfaces of legs and elbows |
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When do qualitative platelet disorders present?
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Childhood
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What is usually the cause of acquired qualitative platelet disorders?
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Drugs
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How are bleeding time, platelet count, coagulation tests, and aggregation tests affected in platelet function disorders?
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Prolonged bleeding time
Normal platelet count Normal coagulation tests (PT, PTT) Abnormal aggregation tests |
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What tests are used to diagnose qualitative platelet disorders?
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Bleeding time (not great)
Aggregation tests |
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What kind of platelet disorder is Bernard-Soulier syndrome? (Qualitative, quantitative, inherited, acquired)
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Qualitative
Inherited |
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How is Bernard-Soulier syndrome inherited?
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Autosomal recessive
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What is the pathophysiological problem that causes Bernard-Soulier syndrome?
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Defective GPIb platelet receptor causing:
- Deficient vWF binding - Defective adhesion |
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How is aggregation affected in Bernard-Soulier syndrome when subjected to ristocetin and when subjected to other agonists?
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Ristocetin:
- No aggregation Other agonists: - Aggregation |
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How are vWF levels in Bernard-Soulier syndrome?
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Normal
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How is bleeding time affected in Bernard-Soulier syndrome?
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Prolonged
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Where does bleeding normally occur in Bernard-Soulier syndrome?
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Mucocutaneous
Nosebleeds |
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How is Bernard-Soulier syndrome treated?
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Transfuse with normal platelets
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What kind of platelet disorder is Glanzmann's thrombasthenia? (Qualitative, quantitative, inherited, acquired)
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Qualitative
Inherited |
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How is Glanzmann's thrombasthenia inherited?
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Autosomal recessive
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What is the pathophysiological problem causing Glanzmann's thrombasthenia?
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Defective GPIIb / IIIa platelet receptor causing:
- No fibrinogen binding - No aggregation |
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How does an aggregation test with ristocetin for a patient with Glanzmann's thrombasthenia appear?
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Aggregation seen
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What are symptoms of Glanzmann's thrombasthenia?
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Mucocutaneous bleeding
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How is bleeding time affected by Glanzmann's thrombasthenia?
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Prolonged
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How is platelet count affected in Glanzmann's thrombasthenia?
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Normal
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How is Glanzmann's thrombasthenia treated?
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Transfuse normal platelets
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What kind of platelet disorder is grey platelet syndrome? (Qualitative, quantitative, inherited, acquired)
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Qualitative
Inherited |
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What is the main qualitative problem with platelets seen in grey platelet syndrome?
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Defects in:
- Dense granules - α-granules |
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How do platelets appear in grey platelet syndrome?
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Enlarged
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How is aggregation affected in grey platelet syndrome?
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No second wave of aggregation
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What usually causes acquired qualitative platelet disorders?
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Drugs
Medical illness |
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How do aspirin and NSAIDs affect platelets?
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Inhibit prostaglandin synthesis:
- Inhibit granule release and activation |
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How does clopidogrel affect platelets?
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Inhibits ADP binding:
- Inhibits aggregation |
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How do anti-GPIIb / IIIa inhibitors affect platelets?
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Inhibit aggregation
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How do PAR1 inhibitors affect platelets?
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Inhibit thrombin binding:
- Inhibit activation |
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What are some medical conditions that can cause acquired qualitative platelet disorders?
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Renal failure
Multiple myeloma |
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How can multiple myeloma affect platelet function?
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Inhibit adherence and aggregation via antibody binding
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How are qualitative, acquired platelet disorders treated?
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Hold drugs
Platelet transfusion |
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What are the three main processes that can cause too few platelets?
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Decreased platelet production
Abnormal platelet distribution Increased platelet destruction |
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What kind of platelet condition is May-Hegglin anomaly? (Quantitative, qualitative, inherited, acquired)
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Quantitative
Inherited |
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How do platelets appear in an instance of May-Hegglin anomaly?
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Enlarged
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What kind of platelet condition is Wiskott-Aldrich syndrome? (Qualitative, quantitative, inherited, acquired)
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Quantitative
Inherited |
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What causes the decreased platelet levels seen in Wiskott-Aldrich syndrome?
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Abnormal signalling in hematopoietic cells
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How are quantitative, inherited platelet abnormalities diagnosed?
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Seen in childhood
Bone marrow test |
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What can cause acquired, quantitative platelet disorders?
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Infiltrative bone marrow disease
Viral infection Drugs Nutritional deficiencies |
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How are acquired, quantitative platelet disorders treated?
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Platelet transfusion (if bleeding)
Treat underlying cause |
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Where is a common site that platelets be abnormally distributed?
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Spleen
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Are syndromes that involve an increased destruction in platelets generally inherited or acquired?
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Acquired
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What are the two major subsets of conditions that involve the increased destruction of platelets?
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Immune
Non-immune |
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What type of platelet condition is autoimmune thrombocytopenic purpura (ITP)? (Qualitative, quantitative, inherited, acquired)
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Acquired
Quantitative |
