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Myeloproliferative neoplasms (MPNs) are a group of rare blood cancers in which excess red blood cells, white blood cells or platelets are produced in the bone marrow. Myelo refers to the bone marrow , proliferative describes the rapid growth of blood cells and neoplasm describes that growth as abnormal and uncontrolled.
In hematology, essential thrombocythemia (ET) is a rare chronic blood cancer (myeloproliferative neoplasm) characterised by the overproduction of platelets (thrombocytes) by megakaryocytes in the bone marrow. [3] It may, albeit rarely, develop into acute myeloid leukemia or myelofibrosis. [3]
The myeloid neoplasms contain acute and chronic leukemias, myelodysplastic syndromes (MDSs) and myeloproliferative neoplasms (MPNs). MPNs are characterised by increased production of myeloid blood cells, with a higher than normal number of mature cells.
It was characterised as a myeloproliferative condition in 1951 by William Dameshek. [37] [38] The disease was also known as myelofibrosis with myeloid metaplasia and agnogenic myeloid metaplasia [39] The World Health Organization utilized the name chronic idiopathic myelofibrosis until 2008, when it adopted the name of primary myelofibrosis.
It is a type of myeloproliferative neoplasm associated with a characteristic chromosomal translocation called the Philadelphia chromosome. CML is largely treated with targeted drugs called tyrosine-kinase inhibitors (TKIs) which have led to dramatically improved long-term survival rates since 2001.
Once the reactive causes of thrombocythemia are ruled out, clonal thrombocythemia should be considered. The most common cause of clonal thrombocythemia is a myeloproliferative neoplasm. These include: essential thrombocythemia, chronic myelogenous leukemia, polycythemia vera, and primary myelofibrosis. [8]
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