Before we jump into the details of essential thrombocythemia (ET), let’s back up for a little perspective. ET is one of a group of disorders called chronic myeloproliferative disorders. These disorders share a lot of similarities: they occur in adults, they have a relatively good prognosis, and they’re caused by mutations in proteins involved in cell growth. The main thing that defines all of them is this: they have malignant, maturing myeloid cells filling up the bone marrow and spilling out into the blood.
The predominant type of proliferating myeloid cell is different in each disorder, though. In ET, the megakaryocytes are proliferating more than any of the other myeloid cell lines. These megakaryocytes make platelets, of course, which spill over into the blood—so the blood has tons and tons of platelets in it. Bottom line: essential thrombocythemia is a chronic myeloproliferative disorder characterized by massive platelet production.
After listening to this AudioBrick, you should be able to:
- Describe the characteristic blood and bone marrow findings in essential thrombocythemia.
- Describe the typical presentation and clinical course of essential thrombocythemia.
- Explain why essential thrombocythemia is a diagnosis of exclusion.
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