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Glossary of 6. Essential thrombocythemia

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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?
-Problems causesd by overactive platelets in circulation

-Primary megakaryo abnormalities
what problems do platelets cause?
-Lo-grade intravascular clotting
-Immune complexes
-Leukoproteins
-Abnormal blood flow
What are the primary meg abnormalities?
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?
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?
-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?
-Meg hyperplasia, increased platelets/clumps.
-Erythroid/myeloid hyperplasia
-Iron stores are fine.
What does the Pb smear show in ET?
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.

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