Vol 3, No 3 (2012)
Review paper
Published online: 2012-10-11
Differential diagnosis of essential thrombocythemia
Hematologia 2012;3(3):201-210.
Abstract
Essential thrombocythemia (ET) is a chronic myelopoliferative neoplasm that involves
primarily the megakaryocytic lineage. Thrombocytosis is a common hematologic finding and
the ET lacks of specific genetic or biologic hallmark therefore the differential diagnosis includes
several reactive and neoplastic megakariocytic proliferations. Similar laboratory findings
(thrombocytosis not associated with leucocytosis, polycythemia nor anemia) as well as cytoand
histologic characteristics (proliferation of the megakaryocytic lineage, without significant
fibrosis, neutrophil granulopoiesis nor erythropoiesis) make difficult to distinct ET from the
pre-polycythemic stage of polycythemia vera and the prefibrotic and early stage of primary
mielofibrosis. An evaluation of the bone marrow biopsy meeting 2008 World Health
Organization (WHO) diagnostic criteria for myeloproliferative neoplasms complying with distinctive megakariocyte morphology and distribution is diagnostic necessity. The differential
diagnosis of ET is clinically important because of distinct treatment options, prognosis and
disease progression such as myelofibrosis or transformation into acute myeloid leukemia.
primarily the megakaryocytic lineage. Thrombocytosis is a common hematologic finding and
the ET lacks of specific genetic or biologic hallmark therefore the differential diagnosis includes
several reactive and neoplastic megakariocytic proliferations. Similar laboratory findings
(thrombocytosis not associated with leucocytosis, polycythemia nor anemia) as well as cytoand
histologic characteristics (proliferation of the megakaryocytic lineage, without significant
fibrosis, neutrophil granulopoiesis nor erythropoiesis) make difficult to distinct ET from the
pre-polycythemic stage of polycythemia vera and the prefibrotic and early stage of primary
mielofibrosis. An evaluation of the bone marrow biopsy meeting 2008 World Health
Organization (WHO) diagnostic criteria for myeloproliferative neoplasms complying with distinctive megakariocyte morphology and distribution is diagnostic necessity. The differential
diagnosis of ET is clinically important because of distinct treatment options, prognosis and
disease progression such as myelofibrosis or transformation into acute myeloid leukemia.
Keywords: essential thrombocythemia2008 WHO classificationdifferential diagnosis
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