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Polycythemia is defined as serum hematocrit (Hct) or hemoglobin (HgB) exceeding normal ranges expected for age and sex, typically Hct >49% in healthy adult men and >48% in women, or HgB >16.5 g/dL in men or >16.0 g/dL in women. [8] The definition is different for neonates and varies by age in children. [9] [10]
In oncology, polycythemia vera (PV) is an uncommon myeloproliferative neoplasm in which the bone marrow makes too many red blood cells. [1] The majority of cases [ 2 ] are caused by mutations in the JAK2 gene, most commonly resulting in a single amino acid change in its protein product from valine to phenylalanine at position 617.
Polycythemia vera (PV) is associated most often with the JAK2 V617F mutation greater than 95% of cases, whereas the remainder has a JAK2 exon 12 mutations. High hemoglobin or hematocrit counts are required, as is a bone marrow examination showing "prominent erythroid , granulocytic and megakaryocytic proliferation with pleomorphic, mature ...
Polycythemia vera (PV), a myeloproliferative disorder in which the bone marrow produces excessive numbers of red cells, is associated with elevated hematocrit. [citation needed] Chronic obstructive pulmonary disease (COPD) and other pulmonary conditions associated with hypoxia may elicit an increased production of red blood cells.
Plethora in the newborn, also known as polycythemia of neonates is an increase in the blood volume due in large part to the late clamping of the umbilical cord. [5] This can cause transient tachypnea of the newborn a temporary breathing problem.
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An increase in the ratio of RBCs to plasma volume, as seen in the setting of polycythemia and hypovolemia, decreases rouleaux formation and decreases sedimentation. Rouleaux formation is retarded by albumin proteins.
Currently, the most common cause is displacement of bone marrow by metastatic cancer (extramedullary hematopoiesis tends to be modest). Other causes include myeloproliferative disorders (especially late-stage or spent polycythemia vera), granulomatous diseases, and (lipid) storage diseases. Myelofibrosis can occur in all of these.
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