Myeloproliferative Disorders and the Hyperviscosity Syndrome

Adams, Bruce D.; Baker, Russell; Lopez, J. Abraham; Spencer, Susan S.

doi:10.1016/j.emc.2009.04.001

Cited by 44 publications

(34 citation statements)

References 91 publications

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“…Leukocytosis, including neutrophilia, correlates with an increased risk of thrombosis in patients with MPNs (35,36). An increased RBC mass in these patients also is well known to be contributory (37). Although loss of Plek2 normalized the total leukocyte count (including the absolute neutrophil count), depletion of leukocytes by Ly-6G injection in JAK2 V617F -knockin mice did not affect vascular occlusion or lethality.…”

Section: Plek2mentioning

confidence: 99%

Loss of pleckstrin-2 reverts lethality and vascular occlusions in JAK2V617F-positive myeloproliferative neoplasms

Zhao¹,

Mei²,

Cao³

et al. 2017

Journal of Clinical Investigation

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Section: Plek2mentioning

confidence: 99%

Loss of pleckstrin-2 reverts lethality and vascular occlusions in JAK2V617F-positive myeloproliferative neoplasms

Zhao¹,

Mei²,

Cao³

et al. 2017

Journal of Clinical Investigation

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“…The prothrombotic mechanisms of an elevated hematocrit (HCT) have been clearly demonstrated in PV. 21 An elevated HCT level can increase the thrombotic risk by multiple pathways (Figure 2). Under the low shear rates, as in the venous bed, a major thrombotic role is played by hyperviscosity, while at high shear rates, the raise of red cell mass displaces platelets toward the Figure 1.…”

Section: Principal Hemostatic Abnormalities Of Mpn Blood and Vascularmentioning

confidence: 99%

Myeloproliferative neoplasms and thrombosis

Barbui¹,

Finazzi²,

Falanga

2013

Blood

344

302

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Major causes of morbidity and mortality in myeloproliferative neoplasms are represented by arterial and venous complications, progression to myelofibrosis, and transformation to acute leukemia. The pathogenesis of thrombosis results from a complex interplay of clinical and disease-related factors. Abnormalities of blood cells arising from the clonal proliferation of hematopoietic stem cells involve not only quantitative changes but also qualitative modifications that characterize the switch of these cells from a resting to a procoagulant phenotype. According to age and previous thrombosis, patients are classified in a “high risk” or “low risk”. Novel disease-related determinants such as leukocytosis and JAK2V617F mutational status and/or mutational burden are now under active investigation. In low-risk polycythemia vera patients, only phlebotomy and primary antithrombotic prophylaxis with aspirin is recommended, while in high-risk patients cytotoxic therapy is considered. Whether novel drugs targeting the constitutively active JAK2/STAT pathway will improve the management of thrombosis is a challenge for future studies.

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“…Enhanced blood viscosity due to an elevated hematocrit provokes thrombogenic blood flow disturbances in PV. At high shear rates, platelets are displaced near the vessel wall, which facilitates their shear-induced activation (14). Platelet activation is also a hallmark of ET, as indicated by increased expression of P-selectin, an inflammatory adhesion molecule stored inside platelet α-granules, and platelet-leukocyte conjugate formation.…”

Section: Discussionmentioning

confidence: 99%