Disease: Polycythemia Vera (PV) Phenotype / cell stem origin: The disease is a chronic myeloproliferative disorder originating from a mutated pluripotent stem cell capable of producing red blood cells, granulocytes and megakaryocytes. Polycythemia vera: stem-cell and probable clonal origin of the disease. Polycythaemia vera (PV) belongs to the group of Philadelphia chromosome-negative myeloproliferative neoplasms. Derived from the Greek words erythros ("red"), melos ("limb") and algos ("pain"); first used in 1878 by Silas Weir Mitchell to describe a syndrome of red congestion and burning pain in the hands and feet. Learn more here. Polycythemia vera. One study indicated that there were 65,243 patients with PV in the United States in 2003. Adamson JW, Fialkow PJ, Murphy S, et al. Virtually all patients with PV harbor a mutation in the nonreceptor tyrosine kinase JAK2, with the majority of patients harboring the classic JAK2. Polycythemia vera is a hematopoietic stem cell disease of clonal origin. Polycythemia vera is a rare condition. It can occur due to rare underlying conditions or certain situations, such as high altitudes. Polycythemia vera is typically associated with an elevated white blood cell count (leukocytosis) and platelet count (thrombocytosis). Cryptogenic (KRIP-to-JEN-ik) polycythemia Erythremia (ER-ih-THRE-me-ah) Erythrocytosis (eh-RITH-ro-si-TO-sis) megalosplenica (MEG-ah-lo-SPLE-ne-kah) Myelopathic (MY-e-lo-PATH-ik) polycythemia Myeloproliferative (MY-e-lo-pro-LIF-er-ah-tiv) disorder Osler disease Polycythemia rubra vera Polycythemia with chronic cyanosis (SI-ah-NO-sis) Primary polycythemia Splenomegalic (SPLE-no-me … Iron overload: People with thalassemia can get an overload of iron in their bodies, either from the disease itself or from frequent blood transfusions.Too much iron can result in damage to the heart, liver, and endocrine system, which includes glands that produce hormones that regulate processes throughout the body.The damage is characterized by excessive deposits of iron. Two patients in the series of Erf (1956) were brothers and 3 others had 'a definite family history.' Erythromelalgia in a 77-year-old woman with longstanding polycythemia vera. Its most prominent feature is an elevated absolute red blood cell mass because of uncontrolled red blood cell production. Modan (1965) suggested that in only 2 reports of familial PV was the diagnosis completely documented (Lawrence and Goetsch, 1950; Erf, 1956). There is a malignant transformation of the multipotential stem cell with excess production of red cells. Polycythemia Rubra Vera (PRV) or Primary Polycythemia Vera is a chronic myeloproliferative disorder characterized by an increase in RBC mass, which often manifests as an increased hematocrit (Hct). In some cases, B-lymphocyte involvement by the clonal proliferation was documented; T-lymphocytes are rarely involved by the malignant process. Polycythemia is a condition in which the body produces too many red blood cells. Polycythemia vera is a disease of the bone marrow and leads to an increased number of red blood cells. PRV involves increased production of all cell lines, including RBCs, … Polycythemia vera (PV) is a stem cell disorder characterized as a panhyperplastic, malignant, and neoplastic marrow disorder. Initial results from the Polycythemia Rubra Study Group suggest that therapy with chlorambucil is associated with an unacceptably high risk for development of acute leukemia, and 32P is preferred for situations in which phlebotomy alone is insufficient. It is a clonal haematopoietic disorder characterised clinically by erythrocytosis and often thrombocytosis, leukocytosis, and splenomegaly. Etymology . Vitamin B-12, also known as cobalamin, is needed for red blood cell production. Polycythemia vera is a myeloproliferative neoplasm characterized by clonal hematopoiesis and an absolute increase in the red blood cell mass, with an associated leukocytosis and thrombocytosis. Correcting a vitamin B-12 deficiency can unmask the symptoms of polycythemia vera. Owen (1924) emphasized the familial nature of polycythemia vera and presented a possible example. Lawrence and Goetsch (1950) described 3 affected sibs.