The vascular complications of polycythemia

Norman, I L; Allen, Edgar V.

doi:10.1016/s0002-8703(37)91050-0

Cited by 66 publications

(6 citation statements)

References 14 publications

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“…Presenting circulatory disturbances of the extremities in patients with PV 8-15 16-24 are described as burning painful and ulcerating toes, features of erythromelalgia, acrocyanosis of burning type, ischemic attacks of digital arteries, and peripheral gangrene with palpable arterial pulsations. [8][9][10][11][12][13][14][15] Smith and Allen 15 first described that aspirin promptly relieved erythromelalgic pain for $ 3 days. This long-lasting effect of a single dose of aspirin is so specific for erythromelalgia that it can be used as a diagnostic criterion.…”

Section: Aspirin-sensitive Erythromelalgia In Et and Pvmentioning

confidence: 99%

“…We could demonstrate that erythromelalgia, with its acrocyanotic ischemic complications (atypical and typical transient cerebral, ocular, and coronary ischemic attacks) are caused by plateletmediated thrombosis in the end-arterial circulation. [6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23][24] Recent data provide good evidence that the first-line treatment option in ET and PV is prevention of minor and major thrombotic complications by control of platelet function with low-dose aspirin in ET and PV, and control of hematocrit by phlebotomy alone in the majority of PV patients. Clear indication for the control of high platelet counts, leukocytosis, and/or splenomegaly by anagrelide, interferon, and/or hydroxyurea (HU) is needed in a minority of ET and PV patients with a high thrombohemorrhagic risk or progressive MPD.…”

mentioning

confidence: 99%

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Clinical and Laboratory Features, Pathobiology of Platelet-Mediated Thrombosis and Bleeding Complications, and the Molecular Etiology of Essential Thrombocythemia and Polycythemia Vera: Therapeutic Implications

Michiels

Berneman

Bockstaele

et al. 2006

Semin Thromb Hemost

110

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Microvascular disturbances in essential thrombocythemia (ET) and polycythemia vera (PV), including erythromelalgia, and atypical and typical transient cerebral, ocular, and coronary ischemic attacks, are caused by platelet-mediated transient and occlusive thrombosis in the end-arterial circulation. ET patients with microvascular disturbances have shortened platelet survival, increased beta-thromboglobulin (beta-TG), platelet factor 4 (PF4), and thrombomodulin (TM) levels, and increased urinary thromboxane B2 (TXB2) excretion, indicating platelet-mediated thrombotic processes. Inhibition of platelet cyclooxygenase-1 by aspirin is followed by relief of microvascular disturbances; correction of shortened platelet survival; correction of increased plasma beta-TG, PF4, and TM levels; and correction of increased TXB2 excretion to normal. In PV associated with thrombocythemia, increased hematocrit and whole blood viscosity aggravate the platelet-mediated microvascular syndrome of thrombocythemia to produce major arterial and venous thrombotic complications. Correction of hematocrit to normal by phlebotomy will reduce the major arterial and venous thrombotic complications, but fails to prevent the platelet-mediated microvascular circulation disturbances in PV patients because thrombocythemia persists. Complete relief and prevention of microvascular and major thrombosis in ET and PV patients, in addition to phlebotomy, are obtained by treatment with aspirin and not with coumarin. The discovery of JAK2 V617F gain of function mutation in patients with myeloproliferative disorders (MPDs) expands our insights into the molecular etiology and biological features of ET, PV, and chronic idiopathic myelofibrosis (CIMF). The current concept is that heterozygous JAK2 V617F mutation with increased kinase activity is enough for megakaryocyte proliferation and increased hypersensitive platelets with no or slightly increased erythropoiesis in ET and in early PV mimicking ET. Homozygous JAK2 mutation with pronounced kinase activity is associated with trilinear megakaryocyte, erythroid, and granulocytic myeloproliferation, myeloid metaplasia, and secondary myelofibrosis (MF), with the most frequent clinical picture of classical PV complicated by major thrombosis in addition to the platelet-mediated microvascular thrombotic syndrome of thrombocythemia. The positive predictive value of a JAK2 V617F polymerase chain reaction test for the diagnosis of MPDs is high (near to 100%), but only half of ET and MF (sensitivity 50%) and the majority of PV (sensitivity 85 to 97%) are JAK2 V617F positive. Bone marrow histopathology, when used in combination with specific markers such as serum erythropoietin, PRV-1, endogenous erythroid colony formation, peripheral blood parameters and red cell mass, has a high sensitivity and specificity (near 100%) to detect the early and overt stages of the MPDs and to differentiate between ET, PV, and CIMF in both JAK2 V617F-positive and -negative MPDs.

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Section: Aspirin-sensitive Erythromelalgia In Et and Pvmentioning

confidence: 99%

mentioning

confidence: 99%

Clinical and Laboratory Features, Pathobiology of Platelet-Mediated Thrombosis and Bleeding Complications, and the Molecular Etiology of Essential Thrombocythemia and Polycythemia Vera: Therapeutic Implications

Michiels

Berneman

Bockstaele

et al. 2006

Semin Thromb Hemost

110

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“…Patients with polycythemia Vera have a high incidence of coronary occlusion but the overwhelming majority of patients are in the older age group and have underlying atherosclerosis [5, 6,8]. Younger patients with coronary thrombosis have been reported, however [ 5 ] . Thrombosis of large proximal arteries is unusual, and to the best of our knowledge this is the first reported case of polycythemia Vera complicated by acute total occlusion of the aorta.…”

Section: Discussionmentioning

confidence: 99%

Acute total aortic occlusion during cardiac catheterization associated with polycythemia vera

Zinn

Applegate

Walsh

1988

Cathet. Cardiovasc. Diagn.

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“…In PV, between 30 and 50% of patients present with vascular complications. [1][2][3] These occur both in arteries and in veins, approximately equally. 3,4 If patients remain untreated, the median survival is only 18 months with the majority dying of vascular occlusive lesions.…”

Section: Vascular Complications In Polycythemiamentioning

confidence: 99%

Hemorheologic Considerations in the Pathogenesis of Vascular Occlusive Events in Polycythemia Vera

Pearson¹

1997

Semin Thromb Hemost

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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.

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The vascular complications of polycythemia

Cited by 66 publications

References 14 publications

Clinical and Laboratory Features, Pathobiology of Platelet-Mediated Thrombosis and Bleeding Complications, and the Molecular Etiology of Essential Thrombocythemia and Polycythemia Vera: Therapeutic Implications

Clinical and Laboratory Features, Pathobiology of Platelet-Mediated Thrombosis and Bleeding Complications, and the Molecular Etiology of Essential Thrombocythemia and Polycythemia Vera: Therapeutic Implications

Acute total aortic occlusion during cardiac catheterization associated with polycythemia vera

Hemorheologic Considerations in the Pathogenesis of Vascular Occlusive Events in Polycythemia Vera

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