6. Essential thrombocythemia
Terms
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- Essentially what is ET?
- overproduction of platelets; all the other cells are fine.
- What are the 2 main types of problems in ET?
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-Problems causesd by overactive platelets in circulation
-Primary megakaryo abnormalities - what problems do platelets cause?
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-Lo-grade intravascular clotting
-Immune complexes
-Leukoproteins
-Abnormal blood flow - What are the primary meg abnormalities?
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1. Abnormal stem clone causes overprod of ineffective platelets, normals are supprsd.
2. Platelets are less dense b/c of ploidy # increase; granules aren't good. - How do symptoms progress in ET patients?
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Initial: asymptomatic
Later: anemia, hemorrhage, thrombotic episodes, Splenomegaly, death in 5 yrs - What does ET need to be differentiated from?
- Reactive thrombocytosis
- What sorts of lab results indicate ET?
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-V. high plt: 600-2500
-Platelet clumps on PBsmear
Look large, but normal volume.
-Abnormal function in bleeding time/aggregation. - What problems for bone marrow procedure are encountered in ET?
- -Rapid clotting during aspiration b/c of platelets
- what does the BM smear show, if you can get one?
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-Meg hyperplasia, increased platelets/clumps.
-Erythroid/myeloid hyperplasia
-Iron stores are fine. - What does the Pb smear show in ET?
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LOTS OF PLATELETS
-No Ph' chromosome, mild leukocytosis, maybe normal.
-Normal LAP - How is a mkd increase in platelets treated?
- with aspirin to defucnctionalize the thrombotic effect.