THROMBOSIS OF THE CORONARY AND CEREBRAL VESSELS AT THE ONSET OF POLYCYTHEMIA VERA (THE AUTHORS’ OWN OBSERVATION)

DOI: https://doi.org/10.29296/25877305-2019-03-08
Download full text PDF
Issue: 
3
Year: 
2019

Professor L. Kozlova(1), MD; G. Kuchma(1), Candidate of Medical Sciences; N. Sokolova(2); S. Lebedenko(2); Professor G. Bagirova(1), MD 1-Orenburg State Medical University 2-Orenburg Regional Clinical Hospital

This paper describes the clinical case of a 39-year-old woman who had long-term polycy-themia vera and developed thrombosis of various localization without other clinical manifestations and complete blood count changes, which were characteristic of this disease. This pathological condition was suspected only when there were significantly elevated levels of hematocrit, plate-lets, and leukocytes, and supported by additional tests (detection of V617 mutation in the Jak2 gene and low erythropoietin levels).

Keywords: 
polycythemia vera
diagnosis of polycythemia vera
thromboses
inherited thrombo-philia



It appears your Web browser is not configured to display PDF files. Download adobe Acrobat или click here to download the PDF file.

References: 
  1. Kovrigina A.M., Bajkov V.V. Istinnaja politsitemija: novaja kontseptsija diagnostiki i klinicheskie formy // Klin. onkogematol. – 2016; 9 (2): 115–22 [Kovrigina A.M., Baikov V.V. Polycythemia Vera: New Diagnostic Concept and Its Types // Clin. oncohematol. – 2016; 9 (2): 115–22. (in Russ)]. DOI: 10.21320/2500-2139-2016-9-2-115-122.
  2. Melikjan A.L., Subortseva, A.M. Kovrigina i dr. Diagnostika latentnoj istinnoj politsitemii (vzgljad klinitsista) // Ter. arh. – 2016; 7: 25–30 [Melikian A.L., Subortseva I.N., Kovrigina A.M. et al. Diagnosis of latent polycythemia vera: A clinician’s opinion // Therapeutic Archive. – 2016; 7: 25–30. (in Russ)]. DOI: 10.17116/terarkh201688725-30.
  3. Barbui T., Thiele J., Vannucchi A. et al. Rationale for revision and proposed changes of the WHO diagnostic criteria for polycythemia vera, essential thrombocythemia and primary myelofibrosis // Blood Cancer J. – 2015; 5 (8): e337. DOI: 10.1038/bcj.2015.64.
  4. Vannucchi A., Barbui T., Cervantes F. et al. Philadelphia chromosome-negative chronic myeloproliferative neoplasms: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up // Ann. Oncol. – 2015; 26 (5): 85–99. DOI: 10.1093/annonc/mdv203.
  5. Abdulkadyrov K.M., Shuvaev V.A., Martynkevich I.S. Chto nam izvestno ob istinnoj politsitemii (obzor literatury i sobstvennye dannye) // Onkogematologija. – 2015; 10: 28–42 [Abdulkadyrov K.M., Shuvaev V.A., Martynkevich I.S. All we know about polycythemia vera: literature review and own experience // Oncohematology. – 2015; 10: 28–42 (in Russ)]. DOI: 10.17650/1818-8346-2015-10-3-28-42.
  6. Ania B., Suman V., Sobell J. et al. Trends in the incidence of polycythemia vera among Olmsted county, Minnesota residents, 1935–1989 // Am. J. Hematol. – 1994; 47 (2): 89–93. DOI: 10.1002/ajh.2830470205.
  7. Vannucchi A., Antonioli E., Guglielmelli P. et al. Clinical correlates of JAK2V617F presenceor allele burden in myeloproliferative neoplasms: A critical reappraisal // Leukemia. – 2008; 22: 1299–307. DOI: 10.1038/leu.2008.113.
  8. Passamonti F., Elena C., Schnittger S. et al. Molecular and clinical features of the myeloproliferative neoplasm associated with JAK2 exon 12 mutations // Blood. – 2011; 117 (10): 2813–6. DOI: 10.1182/blood-2010-11-316810.
  9. Sajt natsional'nogo gematologicheskogo obschestva (npngo.ru). Klinicheskie rekomendatsii po diagnostike i terapii Ph- negativnyh mieloproliferativnyh zabolevanij (istinnaja politsitemija, essentsial'naja trombotsitemija, pervichnyj mielofibroz (redaktsija 2018 g) [The website of the national Hematology society (npngo.ru). Clinical practice guidelines for the diagnosis and treatment of Ph-negative myeloproliferative diseases (true police-time, essential thrombocythemia, primary myelofibrosis (revision 2018) (in Russ)]. Dostupno na / Availaible at https://npngo.ru/uploads/media_document/286/7aa0933a-3a78-4648-908c-2c413aad066a.pdf
  10. Safuanova G.Sh., Gajsarova G.A., Tsareva E.G. i dr. Istinnaja politsitemija, vyjavlennaja u patsienta s restenozom poverhnostnoj bedrennoj arterii. Klinicheskij sluchaj i obzor literatury // Arhiv' vnutrennej meditsiny. – 2017; 3: 224–7 [Safuanova G.Sh., Gaysarova G.A., Tsareva E.G. et al. Polycythemia vera identified in a patient with restenosis of the superficial femoral artery. Clinical case and review of the literature // Archive of Internal Medicine. – 2017; 3: 224–7 (in Russ)]. DOI: 10.20514/2226-6704-2017-7-3-224-227.
  11. Barbui T., Barosi G., Birgegard G. et al. Philadelphia-Negative Classical Myeloproliferative Neoplasms: CriticalConcepts and Management Recommendations From European LeukemiaNet // JCO. – 2011; 29 (6): 761–70. DOI: 10.1182/blood-2012-07-444067.
  12. Davydkin I.L., Rojtman E.V., Kozlova N.S. i dr. Istinnaja politsitemija v sochetanii s arterial'noj gipertoniej: risk razvitija tromboticheskih oslozhnenij // Tromboz gemostaz i reologija. – 2017; 2: 23–30 [Davydkin I.L., Roitman E.V., Kozlova N.S. et al. Polycythemia vera in combination with arterial hypertension: risk of thrombotic complications development // Thrombosis, hemostasis and rheology. – 2017; 2: 23–30 (in Russ)]. DOI: 10.25555/THR.2017.2.0780.
  13. Landolfi R., Cipriani M., Novarese L. Thrombosis and bleeding in polycythemia vera and essential thrombocythemia: Pathogenetic mechanisms and prevention. // Best Pract. Res. Clin. Haematol. – 2006; 19 (3): 617–33. DOI: 10.1016/j.beha.2005.07.011.
  14. Amitrano L., Guardascione M., Ames P. et al. Thrombophilic genotypes, natural anticoagulants, and plasma homocysteine in myeloproliferative disorders: Relationship with splanchnic vein thrombosis and arterial disease // Am. J. Hematol. – 2003; 72 (2): 75–8. DOI: 10.1002/ajh.10254
  15. Shihbabaeva D.I. Znachenie klinicheskih i molekuljarno-geneticheskih faktorov v stratifikatsii riskov u bol'nyh istinnoj politsitemiej. Dis. … kand. med. nauk. SPb, 2017; 113 s. [Shihbabaeva D.I. Significance of clinical and molecular – genetic factors to stratify risk in patients with polycythemia Vera. Dis. ... the candidate of medical Sciences. SPb, 2017; 113 p (in Russ)]. Dostupno na / Available at https://search.rsl.ru/ru/record/01006656930.
  16. Gruppo Italiano Studio Polycythemia Vera: The Natural History of 1213 Patients Followed for 20 Year // Ann. Intern. Med. – 1995; 123 (9): 656–64. DOI: 10.7326/0003-4819-123-9-199511010-00003.
  17. Tanashjan M.M., Kuznetsova P.I., Lagoda O.V. i dr. Mieloproliferativ-
  18. nye zabolevanija i ishemicheskij insul't // Ann. klin. i eksperim. nevrol. – 2014; 8 (2): 41–5 [Tanashyan M.M., Kuznetsova P.I., Lagoda O.V. et al. Myeloproliferative diseases and ischemic stroke // Ann. Clin. Experim. Neurol. – 2014; 8 (2): 41–5 (in Russ)]. Dostupno na / Available at www.annaly–nevrologii.ru
  19. Alvarez-Larran A., Anchocea A., Angona A. et al. Red cell mass measurement in patients with clinically suspected diagnosis of polycythemia vera or essential thrombocythemia // Haematologica. – 2012; 97: 1704–7. DOI: 10.1002/ajh.23585.
  20. Di Nisio M., Barbui T., Di Gennaro L. et al. The haematocrit and platelet target in polycythemia vera // Br. J. Haematol. – 2007; 136: 249–59. DOI: 10.1111/j.1365-2141.2006.06430.x.
  21. Marchioli R., Finazzi G., Specchia G. et al. Cardiovascular events and intensity of treatment in polycythemia vera // N. Engl. J. Med. – 2013; 368 (1): 22–33. DOI: 10.1056/NEJMoa1208500.