Exclusion of Chronic Idiopathic Myelofibrosis

Differentiation of ET from CIMF can occasionally present difficulties, since CIMF may present in a cellular phase with thrombocytosis and ET may undergo myelofibrotic transformation. Features that support CIMF are splenomegaly, typically larger than seen in ET, and peripheral blood leukoerythroblas-tic change and teardrop poikilocytosis. Marrow histology holds a key diagnostic position in distinguishing the cellular phase of CIMF from ET (Table 10-3). Currently, there is not total agreement on the differentiation of ET from a prefibrotic form of CIMF, but a small proportion of patients diagnosed with ET on other grounds do have the changes as given in Table 10-3 of an early form of CIMF. This may have significance in their disease evolution.

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