[Polycythemic hyperviscosity syndromes]. 1983

S Tura, and C Finelli, and P Ricci

Packed red cell volume (VPRC) and total blood volume chiefly affect oxygen transport to tissues and produce the syndrome of polycythemic hyperviscosity. Several studies have demonstrated that a raised VPRC increases the blood viscosity and, under a measured pressure, decreases the flow rate in a capillary tube. Reduced flow results in a less effective tissue perfusion. The oxygen transport at different values of VPRC is shown by an inverted arc-like curve: at normal VPRC levels the oxygen transport is optimal. Hypervolemia affects oxygen transport to tissues: at the same VPRC values, hypervolemic patients have a better oxygen transport. In polycythemia vera (PV), hypervolemia may partially reduce the damage due to the increased blood viscosity. However, in some local areas where fixed vessel diameter (from arteriosclerosis) limits the increased blood flow, hyperviscosity may result in a local tissue ischemia. A relative iron deficiency with associated microcytosis due to venesection frequently occurs in PV. It is also associated with a delayed red blood cell filtration which may contribute to hyperviscosity. When thrombocytosis is present, the risk of thrombotic complications is furthermore increased. We report symptoms and signs at the onset in a series of 80 patients affected by PV. All patients have a minimum follow-up of 4 years. We also report the course of the illness, the treatment effectiveness (venesection, dibromomannitol) on clinical symptoms, the survival curve with analysis of prognostic factors at the onset and the causes of death.

UI MeSH Term Description Entries
D008297 Male Males
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D008954 Models, Biological Theoretical representations that simulate the behavior or activity of biological processes or diseases. For disease models in living animals, DISEASE MODELS, ANIMAL is available. Biological models include the use of mathematical equations, computers, and other electronic equipment. Biological Model,Biological Models,Model, Biological,Models, Biologic,Biologic Model,Biologic Models,Model, Biologic
D010101 Oxygen Consumption The rate at which oxygen is used by a tissue; microliters of oxygen STPD used per milligram of tissue per hour; the rate at which oxygen enters the blood from alveolar gas, equal in the steady state to the consumption of oxygen by tissue metabolism throughout the body. (Stedman, 25th ed, p346) Consumption, Oxygen,Consumptions, Oxygen,Oxygen Consumptions
D011086 Polycythemia An increase in the total red cell mass of the blood. (Dorland, 27th ed) Erythrocytosis,Erythrocytoses,Polycythemias
D011087 Polycythemia Vera A myeloproliferative disorder of unknown etiology, characterized by abnormal proliferation of all hematopoietic bone marrow elements and an absolute increase in red cell mass and total blood volume, associated frequently with splenomegaly, leukocytosis, and thrombocythemia. Hematopoiesis is also reactive in extramedullary sites (liver and spleen). In time myelofibrosis occurs. Erythremia,Osler-Vaquez Disease,Polycythemia Rubra Vera,Polycythemia Ruba Vera,Primary Polycythemia,Disease, Osler-Vaquez,Erythremias,Osler Vaquez Disease,Polycythemia Ruba Veras,Polycythemia Rubra Veras,Polycythemia, Primary,Polycythemias, Primary,Primary Polycythemias,Ruba Vera, Polycythemia,Ruba Veras, Polycythemia,Vera, Polycythemia Ruba,Vera, Polycythemia Rubra,Veras, Polycythemia Ruba,Veras, Polycythemia Rubra
D011379 Prognosis A prediction of the probable outcome of a disease based on a individual's condition and the usual course of the disease as seen in similar situations. Prognostic Factor,Prognostic Factors,Factor, Prognostic,Factors, Prognostic,Prognoses
D012039 Regional Blood Flow The flow of BLOOD through or around an organ or region of the body. Blood Flow, Regional,Blood Flows, Regional,Flow, Regional Blood,Flows, Regional Blood,Regional Blood Flows
D001809 Blood Viscosity The internal resistance of the BLOOD to shear forces. The in vitro measure of whole blood viscosity is of limited clinical utility because it bears little relationship to the actual viscosity within the circulation, but an increase in the viscosity of circulating blood can contribute to morbidity in patients suffering from disorders such as SICKLE CELL ANEMIA and POLYCYTHEMIA. Blood Viscosities,Viscosities, Blood,Viscosity, Blood
D001810 Blood Volume Volume of circulating BLOOD. It is the sum of the PLASMA VOLUME and ERYTHROCYTE VOLUME. Blood Volumes,Volume, Blood,Volumes, Blood

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