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8 Cards in this Set
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- Back
- 3rd side (hint)
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Bernard Soulier
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Thrombocytopenia & large plts & anemia
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http://www.hematologyatlas.com/
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Hermansky-pudlak
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dense granule problem
only first wave aggregation OCA macrophages w/ ceroid Puerto-Ricans, 10q23 (HPS1) pulmonary fibrosis & granulomatous colitis |
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wiskott Aldrich
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Dense granules absent
plts small XLR, thrombocytopenia-eczema-immunodeficinecy Low IgM, Encapsulated organisms -problems Increased risk heme malignancy Xp11 |
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Platelet disorders - absence of dense granules
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Hermansky-Pudlak
Chediak-Higashi Wiskott-Aldrich |
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Chediak-Higashi
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AR
OCA granulocytes & plts giant inclusion granules problem with phagolysosomes -recurrent bacterial infections |
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alpha granule disorders (gray plt syndrome)
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Aggregation blunted except ADP
pale gray (no alpha granules to stain pink) No CD62 NO PF4 Thrombocytopenia |
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Glanzmann's thrombasthenia
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GPIIb/IIIa deficiency - can't bind fibriongen
AR No PLA1 plt antigen Only aggregate with ristocetin No clot retraction (same as dysfibriongenemia) |
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vWD
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#1 bleeding defect
both plt & coag defect Can't aggregate with ristocetin |
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