Chronic Myeloproliferative Disorders Treatment (PDQ®): Treatment - Health Professional Information [NCI] - General Information About Chronic Myeloproliferative Disorders
The chronic myeloproliferative disorders consist of chronic myelogenous leukemia, polycythemia vera (p. vera), primary myelofibrosis, essential thrombocythemia, chronic neutrophilic leukemia, and chronic eosinophilic leukemia. All of these disorders involve dysregulation at the multipotent hematopoietic stem cell (CD34), with one or more of the following shared features:
Overproduction of one or several blood elements with dominance of a transformed clone.
Hypercellular marrow/marrow fibrosis.
Thrombotic and/or hemorrhagic diatheses.
Extramedullary hematopoiesis (liver/spleen).
Transformation to acute leukemia.
Overlapping clinical features.
Patients with p. vera and essential thrombocythemia have marked increases of red blood cell and platelet production, respectively. Treatment is directed at reducing the excessive numbers of blood cells. Both p. vera and essential thrombocythemia can develop a spent phase late in their courses that resembles primary myelofibrosis with cytopenias and marrow hypoplasia and fibrosis.[1,2,3] A specific point mutation in one copy of the Janus kinase 2 gene (JAK2), a cytoplasmic tyrosine kinase, on chromosome 9, which causes increased proliferation and survival of hematopoietic precursors in vitro, has been identified in most patients with p. vera, essential thrombocythemia, and idiopathic myelofibrosis.[4,5,6,7,8,9] Researchers are pursuing specific targeting of this aberrant protein.
Adult primary liver cancer is a disease in which malignant (cancer) cells form in the tissues of the liver.
The liver is one of the largest organs in the body. It has four lobes and fills the upper right side of the abdomen inside the rib cage. The liver has many important functions, including:
Filtering harmful substances from the blood so they can be passed from the body in stools and urine.
Making bile to help digest fats from food.
Storing glycogen (sugar), which the body uses for...
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WebMD Public Information from the National Cancer Institute
September 04, 2014
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