TY - CHAP M1 - Book, Section TI - Myeloproliferative Disorders A1 - Mascarehas, John O. A1 - Hoffman, Ronald A2 - Murray, Michael F. A2 - Babyatsky, Mark W. A2 - Giovanni, Monica A. A2 - Alkuraya, Fowzan S. A2 - Stewart, Douglas R. PY - 2014 T2 - Clinical Genomics: Practical Applications in Adult Patient Care AB - Disease summary:The classic myeloproliferative neoplasms (MPNs) are a group of clonal hematopoietic stem cell disorders associated with an increased thrombotic tendency and the potential to evolve into acute myeloid leukemia. The central and shared feature in MPNs is effective clonal myeloproliferation with resultant peripheral blood granulocytosis, thrombocytosis, or erythrocytosis that is devoid of dyserythropoiesis, or granulocytic dysplasia. For the sake of this chapter, only the four classic MPNs will be discussed.Chronic myelogenous leukemia (CML) is characterized by a pathogenic reciprocal chromosomal translocation involving chromosomes 9 and 22, t(9;22).Polycythemia vera (PV) is a characterized by an absolute increase in red cell mass and often accompanied by leukocytosis, thrombocytosis, and splenomegaly.Essential thrombocythemia (ET) is characterized primarily by thrombocytosis and an elevated risk for both thrombotic and hemorrhagic consequences.Primary myelofibrosis (PMF) is characterized by progressive symptomatic splenomegaly, cytopenias, peripheral blood leukoerythroblastosis and dacrocytes, bone marrow fibrosis, and extramedullary hematopoiesis.Differential diagnosis:Myelodysplastic syndrome (MDS), acute myeloid leukemia, juvenile and chronic myelomonocytic leukemia, secondary causes of thrombocytosis, erythrocytosis and marrow fibrosis, leukemoid reaction, chronic eosinophilic leukemia, chronic neutrophilic leukemia SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/10/07 UR - accessmedicine.mhmedical.com/content.aspx?aid=1102699917 ER -