Neurological Complications of Myeloproliferative Syndromes with Negative Philadelphia Chromosome (MPS Ph-) in Lome Tertiary Hospital
Clinical Neurology and Neuroscience
Volume 3, Issue 1, March 2019, Pages: 11-15
Received: Feb. 13, 2019;
Accepted: Mar. 15, 2019;
Published: May 27, 2019
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Komi Assogba, Neurology Service, Campus University Hospital, Lomé, Togo
Kodzo Vinyo Kumako, Neurology Service, Campus University Hospital, Lomé, Togo
Kossivi Martin Apetse, Neurology Service, Campus University Hospital, Lomé, Togo
Essohana Justin Padaro, Clinical Hematology Services, Campus University Hospital, Lomé, Togo
Abago Balaka, Internal Medicine Service, Sylvanus Olympio University Hospital, Lomé, Togo
Abdoullaye Idrissou, Neurology Service, Campus University Hospital, Lomé, Togo
Komi Igneza Agbotsou, Neurology Service, Campus University Hospital, Lomé, Togo
Nyenèvi Komla Anayo, Neurology Service, Campus University Hospital, Lomé, Togo
Abdullah Blakime, Neurology Service, Campus University Hospital, Lomé, Togo
Agnon Ayélola Koffi Balogou, Neurology Service, Campus University Hospital, Lomé, Togo
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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.
Myeloproliferative Syndrome, Neurological Complications, Philadelphia Chromosome Negative
To cite this article
Kodzo Vinyo Kumako,
Kossivi Martin Apetse,
Essohana Justin Padaro,
Komi Igneza Agbotsou,
Nyenèvi Komla Anayo,
Agnon Ayélola Koffi Balogou,
Neurological Complications of Myeloproliferative Syndromes with Negative Philadelphia Chromosome (MPS Ph-) in Lome Tertiary Hospital, Clinical Neurology and Neuroscience.
Vol. 3, No. 1,
2019, pp. 11-15.
Copyright © 2019 Authors retain the copyright of this article.
This article is an open access article distributed under the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0/
) which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
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.
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.
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.
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.
Marchioli R. Cardiovascular events and intensity of treatment in polycythemia vera. N Engl J Med. 2013; 368 (1): 22–33.
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.
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.
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.
Campbell PJ, Green AR. The myeloproliferative disorders. N Engl J Med. 2006; 355: 2452-66.
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.
Nangalia J, Grinfeld J, Green AR. Pathogenesis of Myeloproliferative Disorders. Annu Rev Pathol. 2016; 11: 101–26.
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.
Elliott MA, Tefferi A. Thrombosis and haemorrhage in polycythaemia vera and essential thrombocythaemia. Br J Haematol. 2005; 128 (3): 275-90.
Viny AD, Levine RL. Genetics of myeloproliferative neoplasms. Cancer J. 2014; 20 (1): 61–5.
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.
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.
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.
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.
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.
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.
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.
Beauverd Y. Pegylated interferon alpha-2a for essential thrombocythemia during pregnancy: outcome and safety. A case serie. Haematologica. 2016; 101 (5): 182–4.
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.
Birgegard G. The Use of Anagrelide in Myeloproliferative Neoplasms, with Focus on Essential Thrombocythemia. Curr Hematol Malig Rep. 2016; 11 (5): 348–55.
Cervantes F, Dupriez B, Passamonti F. Improving survival trends in primary myelofibrosis: an international study. J Clin Oncol 2012; 30: 2981–7.
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.