Clinical Neurology and Neuroscience

| Peer-Reviewed |

Neurological Complications of Myeloproliferative Syndromes with Negative Philadelphia Chromosome (MPS Ph-) in Lome Tertiary Hospital

Received: 13 February 2019    Accepted: 15 March 2019    Published: 27 May 2019
Views:       Downloads:

Share This Article

Abstract

Introduction: Myeloproliferative syndromes with philadelphia (MPS Ph) chromosome negative are diseases little known in our environment and cause grave neurological sequels. The study aimed to describe the neurological complications of these syndromes. Patients and method: It was a retrospective cross-sectional study carried out on the files of patients follow up or hospitalized in hematology or neurology departments of our tertiary hospital from January, 2008 to December, 2017. The variables analyzed were composed of epidemiological data, clinical signs, treatments used, neurological complications, and evolution. Results: Among 39 patients with MPS Ph negative, 30 (76.9%) had neurological complications at the time of diagnostic. Headaches, dizziness and splenomegaly were the most reported clinical signs in 95.2%, 73.6% and 66.7% respectively. Different types of MPS Ph negative were observed with 21 cases of polycythemia vera, 8 cases of essential thrombocythemia and one case of primary myelofibrosis. The research of Jack2V617F mutation was made in 25 patients (83.3%) and was positive in 15. The neurological complications were marked by peripheral neuropathy (20 cases), cerebral venous thrombosis (15 cases) and ischemic stroke in 11 cases. The average length of stay in hospital was 23.6 days. Concerning the treatment, 96.7% had received antiplatelet therapy and cytoreductive treatment was added in 66.7%. The outcome was marked by the remission of symptoms in 11.1% of cases, 46.7% with sequels and 20% of death. Conclusion: The MPS Ph negative patients are often discovered in late stage of the disease progression with neurological complications. Measures need to be taken to improve the early diagnosis and management of MPS Ph chromosome negative.

DOI 10.11648/j.cnn.20190301.13
Published in Clinical Neurology and Neuroscience (Volume 3, Issue 1, March 2019)
Page(s) 11-15
Creative Commons

This is an Open Access article, distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution and reproduction in any medium or format, provided the original work is properly cited.

Copyright

Copyright © The Author(s), 2024. Published by Science Publishing Group

Keywords

Myeloproliferative Syndrome, Neurological Complications, Philadelphia Chromosome Negative

References
[1] Johansson P, Kutti J, Andreasson B, Safai-Kutti S, Vilen L, Wedel H et al. Trends in the incidence of chronic Philadelphia chromosome negative (Ph-) myeloproliferative disorders in the city of Goteborg, Sweden, during 1983-99. J Intern Med. 2004; 256 (2): 161-5.
[2] Finazzi G. Incidence and risk factors for bleeding in 1104 patients with essential thrombocythemia or prefibrotic myelofibrosis diagnosed according to the 2008 WHO criteria. Leukemia. 2012; 26 (4): 716–9.
[3] Gianelli U, Bossi A, Cortinovis I. Reproducibility of the WHO histological criteria for the diagnosis of Philadelphia chromosome-negative myeloproliferative neoplasms. Mod Pathol 2014; 27: 814–22.
[4] Moulard O, Mehta J, Fryzek J et al. Epidemiology of myelofibrosis, essential thrombocythemia, and polycythemia vera in the European Union. Eur J Haematol 2014; 92: 289–97.
[5] Marchioli R. Cardiovascular events and intensity of treatment in polycythemia vera. N Engl J Med. 2013; 368 (1): 22–33.
[6] Marchioli R, Finazzi G, Landolfi R, Kutti J, Gisslinger H, Patrono C et al. Vascular and neoplastic risk in a large cohort of patients with polycythemia vera. J Clin Oncol. 2005; 23 (10): 2224-32.
[7] Titmarsh GJ, Duncombe AS, McMullin MF et al. How common are myeloproliferative neoplasms? A systematic review and meta-analysis. Am J Hematol 2014; 89: 581–7.
[8] Billot S, Kouroupi EG, Le Guilloux J, Cassinat B, Jardin C, Laperche Th, et al. Neurological disorders in essential thrombocythemia. Haematologica 2011; 96 (12): 1866-9.
[9] Campbell PJ, Green AR. The myeloproliferative disorders. N Engl J Med. 2006; 355: 2452-66.
[10] Vannucchi AM. Philadelphia chromosome-negative chronic myeloproliferative neoplasms: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol. 2015; 26 (5): v85–99.
[11] Nangalia J, Grinfeld J, Green AR. Pathogenesis of Myeloproliferative Disorders. Annu Rev Pathol. 2016; 11: 101–26.
[12] Thoennissen NH, Koeffler HPh. Leukaemic Transformation of Philadelphia-chromosome-negative Myeloproliferative Neoplasms – A Review of the Molecular Background. European Oncology & Haematology, 2011; 7 (1): 59–62.
[13] Elliott MA, Tefferi A. Thrombosis and haemorrhage in polycythaemia vera and essential thrombocythaemia. Br J Haematol. 2005; 128 (3): 275-90.
[14] Viny AD, Levine RL. Genetics of myeloproliferative neoplasms. Cancer J. 2014; 20 (1): 61–5.
[15] Wu Zh, Zhang X, Xu X, Chen Y, Hu T, Kang Zh, et al. The mutation profile of JAK2 and CALR in Chinese Han patients with Philadelphia chromosome-negative myeloproliferative neoplasms. J Hematol Oncol 2014; 7: 48-59.
[16] Benton ChB, Tanaka M, Wilson C, Pierce Sh, Zhou L, Cortes J, et al. Increased likelihood of post-polycythemia vera myelofibrosis in Ph-negative MPN patients with chromosome 12 abnormalities. Leuk Res. 2015; 39 (4): 419–23.
[17] Spivak J. Narrative review on Thrombocytosis, polycythemia vera, and JAK2 mutations: The phenotypic mimiking of chronic myeloproliferation. Ann Intern Med. 2010; 152 (5): 300-6.
[18] Tefferi A, Vaidya R, Caramazza D, Finke C, Lasho T, Pardanani A. Circulating interleukin (IL)-8, IL-2R, IL-12, and IL-15 levels are independently prognostic in primary myelofibrosis: a comprehensive cytokine profiling study. J Clin Oncol. 2011; 29 (10): 1356-63.
[19] Alvarez-Larran A. Risk of thrombosis according to need of phlebotomies in patients with polycythemia vera treated with hydroxyurea. Haematologica. 2017; 102 (1): 103–9.
[20] Thiele J, Kvasnicka HM, Müllauer L. Essential thrombocythemia versus early primary myelofibrosis: a multicenter study to validate the WHO classification. Blood 2011; 117: 5710–8.
[21] Vainchenker W, Leroy E, Gilles L, Marty C, Plo I, Stefan N. Constantinescu. JAK inhibitors for the treatment of myeloproliferative neoplasms and other disorders Research 2018; 7: 82-100.
[22] Beauverd Y. Pegylated interferon alpha-2a for essential thrombocythemia during pregnancy: outcome and safety. A case serie. Haematologica. 2016; 101 (5): 182–4.
[23] Boddu P, Falchi L, Hosing Ch, Newberry K, Bose P, Verstovsek Sr. The role of thrombocytapheresis in the contemporary management of hyperthrombocytosis in myeloproliferative neoplasms: a case-based review. Leuk Res. 2017; 58: 14–22.
[24] Birgegard G. The Use of Anagrelide in Myeloproliferative Neoplasms, with Focus on Essential Thrombocythemia. Curr Hematol Malig Rep. 2016; 11 (5): 348–55.
[25] Cervantes F, Dupriez B, Passamonti F. Improving survival trends in primary myelofibrosis: an international study. J Clin Oncol 2012; 30: 2981–7.
[26] Amy Zh, Amber A, Stephen T. Prognostication in Philadelphia Chromosome Negative Myeloproliferative Neoplasms: A review of the recent literature. Curr Hematol Malig Rep. 2017; 12 (5): 397–405.
Author Information
  • Neurology Service, Campus University Hospital, Lomé, Togo

  • Neurology Service, Campus University Hospital, Lomé, Togo

  • Neurology Service, Campus University Hospital, Lomé, Togo

  • Clinical Hematology Services, Campus University Hospital, Lomé, Togo

  • Internal Medicine Service, Sylvanus Olympio University Hospital, Lomé, Togo

  • Neurology Service, Campus University Hospital, Lomé, Togo

  • Neurology Service, Campus University Hospital, Lomé, Togo

  • Neurology Service, Campus University Hospital, Lomé, Togo

  • Neurology Service, Campus University Hospital, Lomé, Togo

  • Neurology Service, Campus University Hospital, Lomé, Togo

Cite This Article
  • APA Style

    Komi Assogba, Kodzo Vinyo Kumako, Kossivi Martin Apetse, Essohana Justin Padaro, Abago Balaka, et al. (2019). Neurological Complications of Myeloproliferative Syndromes with Negative Philadelphia Chromosome (MPS Ph-) in Lome Tertiary Hospital. Clinical Neurology and Neuroscience, 3(1), 11-15. https://doi.org/10.11648/j.cnn.20190301.13

    Copy | Download

    ACS Style

    Komi Assogba; Kodzo Vinyo Kumako; Kossivi Martin Apetse; Essohana Justin Padaro; Abago Balaka, et al. Neurological Complications of Myeloproliferative Syndromes with Negative Philadelphia Chromosome (MPS Ph-) in Lome Tertiary Hospital. Clin. Neurol. Neurosci. 2019, 3(1), 11-15. doi: 10.11648/j.cnn.20190301.13

    Copy | Download

    AMA Style

    Komi Assogba, Kodzo Vinyo Kumako, Kossivi Martin Apetse, Essohana Justin Padaro, Abago Balaka, et al. Neurological Complications of Myeloproliferative Syndromes with Negative Philadelphia Chromosome (MPS Ph-) in Lome Tertiary Hospital. Clin Neurol Neurosci. 2019;3(1):11-15. doi: 10.11648/j.cnn.20190301.13

    Copy | Download

  • @article{10.11648/j.cnn.20190301.13,
      author = {Komi Assogba and Kodzo Vinyo Kumako and Kossivi Martin Apetse and Essohana Justin Padaro and Abago Balaka and Abdoullaye Idrissou and Komi Igneza Agbotsou and Nyenèvi Komla Anayo and Abdullah Blakime and Agnon Ayélola Koffi Balogou},
      title = {Neurological Complications of Myeloproliferative Syndromes with Negative Philadelphia Chromosome (MPS Ph-) in Lome Tertiary Hospital},
      journal = {Clinical Neurology and Neuroscience},
      volume = {3},
      number = {1},
      pages = {11-15},
      doi = {10.11648/j.cnn.20190301.13},
      url = {https://doi.org/10.11648/j.cnn.20190301.13},
      eprint = {https://download.sciencepg.com/pdf/10.11648.j.cnn.20190301.13},
      abstract = {Introduction: Myeloproliferative syndromes with philadelphia (MPS Ph) chromosome negative are diseases little known in our environment and cause grave neurological sequels. The study aimed to describe the neurological complications of these syndromes. Patients and method: It was a retrospective cross-sectional study carried out on the files of patients follow up or hospitalized in hematology or neurology departments of our tertiary hospital from January, 2008 to December, 2017. The variables analyzed were composed of epidemiological data, clinical signs, treatments used, neurological complications, and evolution. Results: Among 39 patients with MPS Ph negative, 30 (76.9%) had neurological complications at the time of diagnostic. Headaches, dizziness and splenomegaly were the most reported clinical signs in 95.2%, 73.6% and 66.7% respectively. Different types of MPS Ph negative were observed with 21 cases of polycythemia vera, 8 cases of essential thrombocythemia and one case of primary myelofibrosis. The research of Jack2V617F mutation was made in 25 patients (83.3%) and was positive in 15. The neurological complications were marked by peripheral neuropathy (20 cases), cerebral venous thrombosis (15 cases) and ischemic stroke in 11 cases. The average length of stay in hospital was 23.6 days. Concerning the treatment, 96.7% had received antiplatelet therapy and cytoreductive treatment was added in 66.7%. The outcome was marked by the remission of symptoms in 11.1% of cases, 46.7% with sequels and 20% of death. Conclusion: The MPS Ph negative patients are often discovered in late stage of the disease progression with neurological complications. Measures need to be taken to improve the early diagnosis and management of MPS Ph chromosome negative.},
     year = {2019}
    }
    

    Copy | Download

  • TY  - JOUR
    T1  - Neurological Complications of Myeloproliferative Syndromes with Negative Philadelphia Chromosome (MPS Ph-) in Lome Tertiary Hospital
    AU  - Komi Assogba
    AU  - Kodzo Vinyo Kumako
    AU  - Kossivi Martin Apetse
    AU  - Essohana Justin Padaro
    AU  - Abago Balaka
    AU  - Abdoullaye Idrissou
    AU  - Komi Igneza Agbotsou
    AU  - Nyenèvi Komla Anayo
    AU  - Abdullah Blakime
    AU  - Agnon Ayélola Koffi Balogou
    Y1  - 2019/05/27
    PY  - 2019
    N1  - https://doi.org/10.11648/j.cnn.20190301.13
    DO  - 10.11648/j.cnn.20190301.13
    T2  - Clinical Neurology and Neuroscience
    JF  - Clinical Neurology and Neuroscience
    JO  - Clinical Neurology and Neuroscience
    SP  - 11
    EP  - 15
    PB  - Science Publishing Group
    SN  - 2578-8930
    UR  - https://doi.org/10.11648/j.cnn.20190301.13
    AB  - Introduction: Myeloproliferative syndromes with philadelphia (MPS Ph) chromosome negative are diseases little known in our environment and cause grave neurological sequels. The study aimed to describe the neurological complications of these syndromes. Patients and method: It was a retrospective cross-sectional study carried out on the files of patients follow up or hospitalized in hematology or neurology departments of our tertiary hospital from January, 2008 to December, 2017. The variables analyzed were composed of epidemiological data, clinical signs, treatments used, neurological complications, and evolution. Results: Among 39 patients with MPS Ph negative, 30 (76.9%) had neurological complications at the time of diagnostic. Headaches, dizziness and splenomegaly were the most reported clinical signs in 95.2%, 73.6% and 66.7% respectively. Different types of MPS Ph negative were observed with 21 cases of polycythemia vera, 8 cases of essential thrombocythemia and one case of primary myelofibrosis. The research of Jack2V617F mutation was made in 25 patients (83.3%) and was positive in 15. The neurological complications were marked by peripheral neuropathy (20 cases), cerebral venous thrombosis (15 cases) and ischemic stroke in 11 cases. The average length of stay in hospital was 23.6 days. Concerning the treatment, 96.7% had received antiplatelet therapy and cytoreductive treatment was added in 66.7%. The outcome was marked by the remission of symptoms in 11.1% of cases, 46.7% with sequels and 20% of death. Conclusion: The MPS Ph negative patients are often discovered in late stage of the disease progression with neurological complications. Measures need to be taken to improve the early diagnosis and management of MPS Ph chromosome negative.
    VL  - 3
    IS  - 1
    ER  - 

    Copy | Download

  • Sections