Hemorheologic considerations in the pathogenesis of vascular occlusive events in polycythemia vera.
Polycythemia vera (PV) commonly presents with vascular occlusion. Untreated patients have a poor prognosis due to the occurrence of thromboses. Treatment outcomes in PV and thrombotic events in other forms of polycythemia and in primary thrombocythemia lead to the conclusion that both raised packed cell volume (PCV) values and quantitative/qualitative platelet changes play a role in the pathogenesis of these vascular occlusive events. In vitro blood viscosity values are predominantly affected by the PCV, particularly at low shear rates and are thus high in untreated PV. Generally, under physiologic conditions blood flow is not governed by blood viscosity due to a number of factors, such as prevailing high shear rates, red cell axial migration, and adaptive vessel diameter changes. Peripheral blood flow is low in untreated PV. Whereas some untreated patients maintain adequate oxygen transport to the tissues, this may not apply in those with cardiac or local vessel disease. There is evidence that low flow rates predispose to occlusive events. Under pathologic low blood flow or static conditions, the rheologic blood changes of untreated PV, demonstrated in vitro, play a role in producing a more deleterious outcome of an occlusive event. Under blood flow conditions, red cell axial migration occurs. This leaves a plasmatic zone at the vessel wall into which platelets are dispersed and where shearing forces are maximal. These forces lead to platelet activation. Increased PCV values reduce the width of the plasmatic zone and lead to an increased possibility of platelet activation, platelet-platelet contact and platelet-vessel wall interaction, particularly when the platelet count is raised as well. These effects increase the likelihood of the initiation of thrombus formation in PV.
Pearson
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Division of Haematology, United Medical School, Guy's Hospital, London, United Kingdom.
D001792:Blood Platelets / Q000502:physiology; D001809:Blood Viscosity; D004730:Endothelium, Vascular / Q000502:physiology; D004912:Erythrocytes / Q000502:physiology; D018056:Hemorheology; D006801:Humans; D011087:Polycythemia Vera / Q000097:blood* / Q000150:complications; D012039:Regional Blood Flow; D014652:Vascular Diseases / Q000097:blood* / Q000150:complications