Recurrent ischaemic cerebrovascular events as presenting manifestations of myeloproliferative neoplasms

Background and purpose Myeloproliferative neoplasms (MPNs) – polycythemia vera, essential thrombocythemia and primary myelofibrosis – are associated with increased risk for ischaemic cerebrovascular events (ICVEs). Due to their low prevalence, MPNs often remain undiagnosed as the cause of ICVEs. Met...

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Bibliographic Details
Published in:European journal of neurology 2019-06, Vol.26 (6), p.903-e64
Main Authors: Stefanou, M. I., Richter, H., Härtig, F., Wang, Y., Örgel, A., Bender, B., Mengel, A., Ziemann, U., Poli, S.
Format: Article
Language:English
Subjects:
Algorithms
Blood cancer
Brain
Diagnosis
essential thrombocythemia
Etiology
JAK2 mutation
Janus kinase 2
Mutation
Myelofibrosis
myeloproliferative neoplasms
Neoplasms
Neuroimaging
Patients
Polycythemia
Polycythemia vera
primary myelofibrosis
Stroke
Transient ischemic attack
Tumors
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Summary:Background and purpose Myeloproliferative neoplasms (MPNs) – polycythemia vera, essential thrombocythemia and primary myelofibrosis – are associated with increased risk for ischaemic cerebrovascular events (ICVEs). Due to their low prevalence, MPNs often remain undiagnosed as the cause of ICVEs. Methods Case records at the University of Tübingen between 2014 and 2017 were screened to identify patients with MPN‐related ICVEs. Clinical features, brain imaging, laboratory findings, applied treatments and neurological outcomes were assessed. Results In all, 3318 patients with ICVEs were identified, and amongst them 17 patients with MPN‐related ICVEs were included in a retrospective study. In 58% of these patients, ICVEs were the first manifestation of the underlying MPN; 24% presented with transient ischaemic attack and 76% with ischaemic stroke. Potentially concurrent ICVE etiologies were noted in 70% of the patients. The majority (94%) of patients were positive for the JAK2 V617F mutation, whilst in 29% recurrent ICVEs (range two to three) were noted prior to MPN diagnosis. Early MPN diagnosis and management was the only significant prognostic factor for ICVE recurrence (P 
ISSN:1351-5101
1468-1331
DOI:10.1111/ene.13907