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

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.