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diagnostic evaluation of essential thrombocythemia (ET)🚧 施工中

diagnostic evaluation of essential thrombocythemia (ET)

• Peripheral smear: large hypogranular platelets

• BM bx: megakaryocytic hyperplasia; ⊖ Phil chr; rarely minor reticulin fibrosis; nl Fe; if atypical megakaryoctyes or ↑ reticulin, consider pre-PMF (Rx same as ET, but ↑ risk MF)

• Mutations: JAK2 V617F in ~50%; CALR in ~45%; MPL in 5–10%; triple negative <5%

• Patients should not meet WHO criteria for diagnosis of CML, PV, PMF, or MDS

• Check vWF in pts w/ plt>1,000,000 and hold ASA (vide infra) if acquired vWD