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22 Cards in this Set
- Front
- Back
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-Bernard-soulier syndrome
-uremia -DIC -paraproteinemia |
defects of adhesion
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-adhesion defect
-inherited autosomal recessive -hemorrhagic problems in childhood;decrease with age -abn. or lack of GPIb adhesion receptor necessary for plt to bind with vWF |
Bernard soulier syndrome
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-mild/moderate thrombocytopenia
-giant platelets - ^^BT (>20min) -normal clot retraction -PF3 avail= norm -plt adhesion decr. |
Bernard soulier sydnrome
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Plt aggregation:
- ADP norm. -Epinepherine norm -Collagen/thrombin variable -ristocetin- abnormal |
Bernard soulier syndrome
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-Glanzmanns thrombasthenia
-Afibrogenemia |
Defects of primary aggregation
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-Inherited autosomal recessive
-homozygotes have recognizable hemorrhagic disorder -defect in GPIIb/IIIa complex; fibrinogen then cannot bind |
Glanzmanns thrombasthenia
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Clinical symptoms:
-brusing, epistaxis, bleeding from mucous membranes, GI bleeding, menorrhagia -ecchymoses @birth or early years -hemorrhagic severity decreases with age -may require transfusions |
Glanzmanns thrombasthenia
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-plt. ct. normal
-clot retraction abnormal -PF3 decreased -BT increased -plt adhesion abnormal |
Glanzmanns thrombasthenia
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Plt aggregation studies:
-ADP=none -Epinepherine= none -collagen=none -thrombin=none -ristocetin= normal |
Glanzmanns thrombasthenia
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-Abnormal plt secretion
-defective/ decreased alpha or dense granules -defect in receptor mediated signal transduction |
defects of release (secretion)
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-alpha granules synthesized but not stored normally
-early form of granules in megakaryocytes but decrease w/ maturity -inherited autosomal -mild moderate thrombocytopenia |
Grey plt syndrom
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-plts: grey, vacuolated, washed-out
-small # of granules present -normal thromboxane generation -variable ADP, epinepherine, collagen, thrombin |
grey plt syndrome
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-deficiency of dense bodies
-associated with albanism (chediak-higashi syndrome), and immune deficiencies (wiskott- aldrich syndrome) |
Storage pool disease
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-autosomal dominant
-mild/moderate bleeding diathesis -epistaxis, purpura, menorrhagia, post traumatic/post surgical bleeding |
Storage pool disease
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-plt ct normal
- ^BT -collagen abnormal -deficient secondary wave with ADP |
Storage pool disease
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-x-linked immune deficiency
-small plts -triad: thrombocytopenia, reoccurent infections, eczema |
Wiskott- aldrich syndrome
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-albanism
-recurrent infections -hemorrhagic tendencies -dense granule deficiency -giant lysosomes |
chediak-higashi anomaly
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-abnormality of membrane receptors
-aspirin-like defect in aggregation and secreation -thromboxane synthetase deficiency -impaired respsonse to thromboxane A2 -Impaired Calcium mobilization -Impaired liberation of arachiodonic acid |
Abnormal receptor mediated signal transduction and secretion
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Caused by:
-drugs -diet(high in fish=low plt function) -diseases (myloproliferative, uremia) |
Acquired qualitative plt disorder
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-aspirin
-non-steroidal anti inflammatory drugs -cardiovascular drugs -psychotropic drugs |
acquired plt dysfunction drugs
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-uremia
-related to the accumulation of waste products -bleeding can be sever w/widespread echymoses and slow GI bleeding -hemodialysis and peritoneal dialysis to control bleeding -paraproteinemias |
Acquired disorders- diseases
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Clonal hematopoeitic disorders:
-myelodysplastic syndrome -myeloproliferative disorders -acute nonlymphatic disorders |
Acquired disorders-diseases
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