Mental disorders in polycythemia vera (review of clinical cases according to literature data)

Mental disorders in polycythemia vera (review of clinical cases according to literature data)

 

Authors

 

F.V. Orlov

Federal State Budgetary Educational Institution of Higher Education “Chuvash State University

named after I.N. Ulyanov”, Cheboksary, Russian Federation

P.B. Zotov

Federal State Budgetary Educational Institution of Higher Education “Tyumen State Medical University”

of the Ministry of Health of the Russian Federation, Tyumen, Russian Federation

A.V. Golenkov

Federal State Budgetary Educational Institution of Higher Education “Chuvash State University

named after I.N. Ulyanov”, Cheboksary, Russian Federation

 

https://doi.org/10.26617/1810-3111-2025-3(128)-83-94

 

Journal: Siberian Herald of Psychiatry and Addiction Psychiatry. 2025; 3 (128):  83-94.

 

Abstract

Introduction. In the literature, mental disorders (MD) occurring in patients with polycythemia vera (PV) as secondary manifestations have not been sufficiently studied. Objective: to study MD in association with PV based on the analysis of clinical cases, mainly described in the English-language scientific literature, in order to establish the relationship between symptoms, risk factors, and outcomes. Materials and Methods. Search, selection, and systematic analysis of clinical cases (PV with MD) retrieved from the PubMed database to compare treatment outcomes. Keywords for information searchwere: “polycythemia vera”, “mental disorders”. Results. The review is devoted to the analysis of literature data on the materials of published clinical case studies of MD in PV in English. Organic MD is a characteristic mental manifestation of PV. Somatogenic psychopathological symptoms in PV are very diverse. In clinical practice, nonpsychotic MD and psychoses, such as delirium and paranoid, are highly common in patients with PV. Cognitive and anxiety disorders, depressive and manic syndromes are described. The appearance of signs of MD (anxiety, depression, restlessness, irritability, depression, cognitive decline) in patients with PV in the development of the disease indicates disease progression. Elder patients are at risk for developing MD in PV. Although it is difficult to state the cause-and-effect relationship between MD and PV with certainty, their common risk factors, however, the improvement in the condition of patients within a short time after phlebotomy and normalization of the hemoglobin level allow assuming that in these cases PV led to the development of secondary mental disorders. Positive dynamics (improvement of the physical and psychological state, pain reduction, improved prognosis) after cytoreduction indicated the presence of a reversible pathogenetic mechanism causing MD. Conclusion. The identification of secondary, potentially correctable or eliminable factors causing the development of MD is extremely important for preventing iatrogenic harm from unjustified pharmacological treatment with psychotropic drugs and minimizing the risk of delay in diagnosis and treatment. Timely diagnosis and therapy of MD at early stages in patients with PV contribute to the optimization of treatment and rehabilitation measures, prevention of complications, reduction of adverse effects of the disease and disability.

 

Keywords: myelofibrosis, polycythemia vera, essential thrombocythemia, thrombosis, mental disorders (pathogenesis), phlebotomy, cytoreduction.

 

Article (pdf)

 

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Materials  

 

For citation: Orlov F.V., Zotov P.B., Golenkov A.V. Mental disorders in polycythemia vera (review of clinical cases according to literature data). Siberian Herald of Psychiatry and Addiction Psychiatry. 2025; 3 (128): 83-94. https://doi.org/10.26617/1810-3111-2025-3(128)-83-94

 

REFERENCES

  1. Tselibeev BA. Mental disorders in somatic diseases. Moscow: Meditsina, 1972:280 (in Russian).
  2. VybornykhDE, SavchenkoVG. Mental disorders in patients with blood system diseases: aspects of diagnosis and treatment. Clinical Oncohematology. Basic Research and Clinical Practice. 2013;6(4):451-464 (in Russian).
  3. Shpilyukova YA, Smetanina OV, Kolpina AA, Illarioshkin SN. A case of chorea with cognitive impairment associated with polycythemia vera. Neurology, Neuropsychiatry, Psychosomatics. 2023;15(6):101-108. https://doi.org/10.14412/20742711-2023-6-101-108(in Russian).
  4. Prieto JM, Blanch J, Atala J, Carreras E, Rovira M, Cirera E, Gastó C. Psychiatric morbidity and impact on hospital length of stay among hematological cancer patients receiving stem-cell transplantation. J Clin Oncol. 2002; 20:1907-1917. https://doi.org/10.1200/JCO.2002.07.101. PMID: 11919251.
  5. Haber J. Psychosis in polycythemia vera. J Nerv Ment Dis. 1952; 115(6):537-540. https://doi.org/10.1097/00005053-195206000-00005. PMID: 14946546.
  6. Severs M, Boelens HM, Diraoui SB, Schuur J. Chorea and a frontal lobe syndrome: a rare neurological presentation of polycythemia vera; a case report. J Am Geriatr Soc. 2012 Mar;60(3):589-90. https://doi.org/10.1111/j.1532-5415.2011.03850.x. PMID: 22409737.
  7. Melikyan AL, Kovrigina AM, Subortseva IN, Shuvaev VA, Afanasiev BV, Ageeva TA, Baikov VV, Vinogradova OYu, Golenkov AK, Gritsaev SV, Zaritskiy AYu, Kaplanov KD, Lomaia EG, Martynkevich IS, Morozova EV, Pospelova TI, Sokolova MA, Sudarikov AB, Turkina AG, Shatokhin YuV, Savchenko VG. National clinical guidelines for diagnosis and therapy of PH-negative myeloproliferative neoplasms (polycythemia vera, essential thrombocythemia, primary myelofibrosis) (edition 2018). Russian Journal of Hematology and Transfusiology. 2018;63(3):275-315 (in Russian).
  8. Marcellino BK, Hoffman R. Recent advances in prognostication and treatment of polycythemia vera. Fac Rev. 021 Mar 12;10:29. https://doi.org/10.12703/r/10-29. PMID: 33817698; PMCID: PMC8009192.
  9. Tefferi A, Barbui T. Polycythemia vera: 2024 update on diagnosis, risk-stratification, and management. Am J Hematol. 2023 Sep;98(9):1465-1487. https://doi.org/10.1002/ajh.27002. Epub 2023 Jun 26. PMID: 37357958.
  10. Jin C, Pu J, Zhou Z, Chen X, Wu J, Zhang B. Rapidly progressive cognitive impairment: an unusual presentation of cerebral venous thrombosis caused by JAK2 V617F-positive primary myelofibrosis: A case report. Medicine (Baltimore) 2020 Aug 21;99(34):e21757. https://doi.org/10.1097/MD.0000000000021757. PMID: 32846801; PMCID: PMC7447497.
  11. Li L, Zhou M, Wu YQ, Fan WN, Li D. Neuropsychiatric disturbance detecting polycythemia vera myelofibrosis: A case report and literature review. Front Neurol. 2023 Sep 22;14:1253468. https://doi.org/10.3389/fneur.2023.1253468. PMID: 37808487; PMCID: PMC10556491.
  12. Haghighat R, Costa DC, Chesser E. Polycythaemia and agoraphobia. J Behav Ther Exp Psychiatry. 1996 Jun;27(2):149-55. https://doi.org/10.1016/0005-7916(96)00006-7. PMID: 8894913.
  13. Yıldırım YE, Erözeren HI, Gültürk E, Çağlar Tanrıverdi N, Balaban ÖD. Psychiatric manifestations in polycythemia vera: A case of refractory delirium and psychosis responding to hematological treatment. Turk J Haematol. 2025 May 22;42(2):167-169. https://doi.org/10.4274/tjh.galenos.2025.2025.0060.Epub 2025 Apr 29. PMID: 40297865; PMCID: PMC12099474.
  14. Rai R, Pieters T. An unusual psychiatric presentation of polycythemia 'Difficulties lie in our habits of thought rather than in the nature of things' Andre Tardieu. BMJ Case Rep. 2013 Apr 9;2013:bcr2012008215. https://doi.org/10.1136/bcr-2012-008215. PMID: 23576647; PMCID: PMC3644952.
  15. Jianu DC, Jianu SN, Dan TF, Munteanu G, Copil A, Birdac CD, Motoc AGM, Docu Axelerad A, Petrica L, Arnautu SF, Sadik R, Iacob N, Gogu AE. An integrated approach on the diagnosis of cerebral veins and Dural sinuses thrombosis (a narrative review). Life (Basel). 2022 May 11;12(5):717. https://doi.org/10.3390/life12050717. PMID: 35629384; PMCID: PMC9145675.
  16. Coelho RO, Reynaldo LFM, Valente GDCB, Silva LF. Outcome of patient with polycythemia rubra vera and psychiatric symptoms. Hematol Transfus Cell Ther. 2022 Jan-Mar;44(1):116-119. https://doi.org/10.1016/j.htct.2020.06.010. Epub 2020 Aug 2. PMID: 32792261; PMCID: PMC8885362.
  17. Chawla M, Lindesay J. Polycythaemia, delirium and mania. Br J Psychiatry. 1993 Jun;162:833-5. https://doi.org/10.1192/bjp.162.6.833. PMID: 8330115.
  18. Murray D, Hodgson R. Polycythaemia rubra vera, cerebral ischaemia and depression. Br J Psychiatry. 1991 Jun;158:842-4. https://doi.org/10.1192/bjp.158.6.842. PMID: 1873637.
  19. Mazzoli M, Benazzi F. Рolycythaemia and psychotic depression. Br J Psychiatry. 1992 Jan;160:134-5. https://doi.org/10.1192/bjp.160.1.134b. PMID: 1544007.
  20. Bauer M. Absolutely therapy-resistant depression and mixed movement disorder in an unusual case of polycythemia vera. Pharmacopsychiatry. 1995 Mar;28(2):66-8. https://doi.org/10.1055/s-2007-979592. PMID: 7624389.
  21. Di Pollina L, Mulligan R, Juillerat Van der Linden A, Michel JP, Gold G. Cognitive impairment in polycythemia vera: partial reversibility upon lowering of the hematocrit. Eur Neurol. 2000;44(1):57-9. https://doi.org/10.1159/000008194. PMID: 10894997.
  22. Alkemade GM, Willems JM. Polycytemia vera presenting as sudden-onset cognitive impairment. J Am Geriatr Soc. 2008 Dec;56(12):2362-3. https://doi.org/10.1111/j.1532-5415.2008.02021.x. PMID: 19093946.
  23. Kowar M, Wilhelm K, Jacobs AH. Polycythämia vera in der differenzialdiagnostischen Abklärung kognitiver Defizite [Polycythemia vera as a cause for progressive cognitive impairment]. Dtsch Med Wochenschr. 2020 Nov;145(22):1625-1627. German. https://doi.org/10.1055/a-1219-9564. Epub 2020 Nov 3. PMID: 33142329 (in German).
  24. Bai J, Ai L, Zhang L, Yang FC, Zhou Y, Xue Y. Incidence and risk factors for myelofibrotic transformation among 272 Chinese patients with JAK2-mutated polycythemia vera. Am J Hematol. 2015 Dec;90(12):1116-21. https://doi.org/10.1002/ajh.24191. Epub 2015 Nov 17. PMID: 26370613.
  25. García-Cabo C, Fernandez-Dominguez J, Mateos V. Sudden hemichorea and frontal lobe syndrome: a rare presentation of unbalanced polycythaemia vera. BMJ Case Rep. 2018 May 14;2018:bcr2017223867. https://doi.org/10.1136/bcr-2017-223867. PMID: 29764825; PMCID: PMC5961552.
  26. GlininOP, KrymskayaOS, AlievAT. Ideas about POEMS syndrome: a neurologist's view. Academic Journal of West Siberia. 2024;20;2(103):10-14. https://doi.org/10.32878/sibir.24-20-02(103)-10-14 (in Russian).