Myeloproliferative diseases and ischemic stroke

Cover Page

Cite item

Full Text


Myeloproliferative diseases (MPD) comprise a group of clonal pathology resulting from genetic alterations at a stem cell level. WHO (2008) divides MPD into several “classical” forms – polycythemia vera, essential thrombocytemia, and primary myelofibrosis. Haemorheologic and haemostatic disturbances have been widely accepted as a significant (in some cases – primary) cause of acute and/or chronic cerebrovascular disease. A pre-existing MPD may potentiate and accelerate the development of circulatory alterations, including those occurring in the brain tissue, leading to ischemic stroke. The present article comprises a literature review on this uncommon pathology, as well as a clinical presentation of a stroke case in a patient with underlying MPD (Vaquez’ disease).

About the authors

Marine M. Tanashyan

Research Center of Neurology

ORCID iD: 0000-0002-5883-8119

D. Sci. (Med.), Prof., Corresponding member of RAS, Deputy Director for science, Head, 1st Neurological department

Russian Federation, Moscow

Polina I. Kuznetsova

Research Center of Neurology

ORCID iD: 0000-0002-4626-6520

Cand. Sci. (Med.), neurologist, researcher, 1st Neurology department

Russian Federation, Moscow

Olga V. Lagoda

Research Center of Neurology

Author for correspondence.
Russian Federation, Moscow

Alla A. Shabalina

Research Center of Neurology

Russian Federation, Moscow

I. N. Subortseva

Research Center of Hematology, Ministry of Health

Russian Federation, Moscow

A. L. Melikyan

Research Center of Hematology, Ministry of Health

Russian Federation, Moscow


  1. Инсульт: диагностика, лечение, профилактика. Под. ред. З.А Суслиной, М.А. Пирадова. Москва. 2008; 288.
  2. Суслина З.А., Танашян М.М., Ионова В.Г. Ишемический инсульт: кровь, сосудистая стенка, антитромботическая терапия. М., 2005; 248.
  3. Танашян М.М. Гемостаз, гемореология и атромбогенная активность сосудистой стенки в ангионеврологии. Анн. клин. и эксперим. неврол. 2007; 2(1): 29–33.
  4. Arellano-Rodrigo E., Alvarez-Larran A., Reverter J.C. et al. Increased platelet and leukocyte activation as contributing mechanisms for thrombosis in essential thrombocythemia and correlation with the JAK2 mutational status. Haematologica. 2006; 91: 169–175.
  5. Barbui T., Finazzi G., Carobbio A. et al. Development and validation of an International Prognostic Score of thrombosis in World Health Organization-essential thrombocythemia (IPSET-thrombosis). Blood. 2012; 120: 5128–5133.
  6. Brill A., Fuchs T.A., Savchenko A.S. et al. Neutrophil extracellular traps promote deep vein thrombosis in mice. J Thromb Haemost. 2012; 10: 136–144.
  7. Brinkmann V., Zychlinsky A. Beneficial suicide: why neutrophils die to make NETs. Nat Rev Microbiol. 2001; 5: 577–582.
  8. Carobbio A., Thiele J., Passamonti F. et al. Risk factors for arterial and venous thrombosis in WHO-defined essential thrombocythemia: an international study of 891 patients. Blood. 2011; 117: 5857–5859.
  9. Cella G., Marchetti M., Vianello F. et al. Nitric oxide derivatives and soluble plasma selectins in patients with myeloproliferative neoplasms. Thromb Haemost. 2010; 104: 151–156.
  10. De Stefano V., Za T., Rossi E. et al. GIMEMA CMD-Working Party Recurrent thrombosis in patients with polycythemia vera and essential thrombocythemia: incidence, risk factors, and effect of treatments. Haematologica. 2008; 93(3): 372–380.
  11. Di Nisio M., Barbui T., Di Gennaro L. et al. The haematocrit and platelet target in polycythemia vera. Br J Haematol. 2007; 136:249–259.
  12. Falanga A., Marchetti M. Thrombotic disease in the myeloproliferative neoplasms. Hematology. Education Program of the American Society of Hematology 2012. 2012; 571–581.
  13. Goette N.P., Lev P.R., Heller P.G. et al. Monocyte IL-2Ralpha expression is associated with thrombosis and the JAK2V617F mutation in myeloproliferative neoplasms. Cytokine. 2010; 51: 67–72.
  14. Hebbel R.P., Boogaerts M.A., Eaton J.W., Steinberg M.H. Erythrocyte adherence to endothelium in sickle-cell anemia. A possible determinant of disease severity. N Eng J Med. 1980; 302: 992–995.
  15. Jensen M.K., De NullyBrown P., Lund B.V. et al. Increased platelet activation and abnormal membrane glycoprotein content and redistribution in myeloproliferative disorders. Br J Haematol. 2000; 110:116–124.
  16. Kesler A., Ellis M.H., Manor Y. et al. Neurological complications of essential thrombocytosis (ET). Acta Neurol Scand. 2000: 102: 299–302.
  17. Klampfl T., Gisslinger H., Harutyunyan A.S. Somatic Mutations of Calreticulin in Myeloproliferative Neoplasms. N Engl J Med. 2013; 369: 2679–2690.
  18. Nangalia J., Massie C.E., Baxter E.J. et al. Somatic CALR Mutations in Myeloproliferative Neoplasms with Nonmutated JAK2. N Engl J Med. 2013; 369: 2391–2405.
  19. Nazabal E.R., Lopez J.M., Perez P.A., Corral P.R. Chorea disclosing deterioration of polycythaemia vera. Postgrad Med J. 2000; 76:658–659.
  20. Pearson T.C., Path F.R.C. Hemorrheologic considerations in the pathogenesis of vascular occlusive events in polycythaemia vera. Semin Thromb Hemost. 1997; 23: 433–439.
  21. Semeraro F., Ammollo C.T., Morrissey J.H. et al. Extracellular histones promote thrombin generation through platelet-dependent mechanisms: involvement of platelet TLR2 and TLR4. Blood. 2011; 118:1952–1961.
  22. Sozer S., Fie. M.L., Schiano T. et al. The presence of JAK2V617F mutation in the liver endothelial cells of patients with Budd-Chiari syndrome. Blood. 2009; 113: 5246–5249.
  23. Tefferi A. Pathogenesis of myelofibrosis with myeloid metaplasia. J Clin Oncol. 2005; 23(33): 8520–8530.
  24. Tefferi A., Thiele J., Orazi A. et al. Proposals and rationale for revision of the World Health Organization diagnostic criteria for polycythemia vera, essential thrombocythemia, and primary myelofibrosis:Recommendations from an ad hoc international expert panel. Blood. 2007; 110: 1092–1097.
  25. Wautier M.P., Nemer W., Gane P. et al. Increased adhesion to endothelial cells of erythrocytes from patients with polycythemia vera is mediated by laminin alpha S chain and Lu/BCAM. Blood. 2007; 110:894–901.
  26. Wautier J.L., Paton R.C., Wautier M.P. et al. Increased adhesion of erythrocytes to endothelial cells in diabetes mellitus and its relation to vascular complications. N Eng J Med. 1981; 305: 237–242.
  27. Varricchio L., Mancini A., Migliaccio A.R. Pathological interactions between hematopoietic stem cells and their niche revealed by mouse models of primary myelofibrosis. Expert Rev Hematol. Author manuscript; 2009; 2(3): 315–334.

Supplementary files

Supplementary Files

Copyright (c) 2014 Tanashyan M.M., Kuznetsova P.I., Lagoda O.V., Shabalina A.A., Subortseva I.N., Melikyan A.L.

Creative Commons License
This work is licensed under a Creative Commons Attribution 4.0 International License.

СМИ зарегистрировано Федеральной службой по надзору в сфере связи, информационных технологий и массовых коммуникаций (Роскомнадзор).
Регистрационный номер и дата принятия решения о регистрации СМИ: серия ПИ № ФС 77-83204 от 12.05.2022.

This website uses cookies

You consent to our cookies if you continue to use our website.

About Cookies