Recurrent ischaemic cerebrovascular events as presenting manifestations of myeloproliferative neoplasms

Abstract

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 < 0.001). Discussion Evidence is provided that, although rare, MPNs represent an underdiagnosed cause of recurrent ICVEs. High clinical awareness is warranted to identify an underlying MPN in patients presenting with sustained, abnormal blood count findings. Clinical algorithms for prompt MPN diagnosis and initiation of MPN treatment (e.g. cytoreductive therapy, phlebotomy) are required. As MPN management comprises a significant protective factor against ICVE recurrence, induction of MPN treatment should be regarded as an integral component of secondary stroke prevention in MPN‐associated ICVEs.

Frequency and predictors of occult cancer in ischemic stroke: A systematic review and meta-analysis

“…[10][11][12] Of the 26 studies in the meta-analyses, only four included non-IS, in proportions of 6.2-15.8%. [15][16][17][18] The meta-analyses included a total of 46,633 individuals with stroke (per study: median, 1,132; IQR: 407-3,069), of which about 0.5% (n ¼ 226) were nonischemic.…”

Prefibrotic Myelofibrosis Presenting with Multiple Cerebral Embolic Infarcts and the Rare MPL W515S Mutation

“…As MPN are relatively rare, it is probable they represent an underdiagnosed cause of cerebral thrombosis. High 2 Case Reports in Hematology clinical awareness is warranted to identify an underlying MPN in order that a prompt treatment is initiated (e.g., antiplatelet, cytoreductive, and phlebotomy) [24,25]. While some studies have suggested routine screening for JAK2 V617F in patients presenting with stroke and/or cerebral venous thrombosis (CVT) [26], given the lower risk of thrombosis in CALR-and MPL-mutated MPN patients and the near absence of these mutations in CVT cohorts [27,28], wider evaluation of mutation status might be reserved for those patients with sustained abnormal blood count findings.…”

Characteristics of arterial stroke in myeloproliferative neoplasms: A French monocentric study

“…In a retrospective study, among 3318 patients with ischemic vascular cerebral events, 17 (0.5%) were diagnosed with MPN, and stroke was the revealing manifestation for 58% of them. 1 Moreover, 29% of these patients had a history of repeated ischemic cerebral event preceding the diagnosis of MPN. Nevertheless, in this large cohort, another associated cause existed in all these stroke patients with MPN.…”

“…Nevertheless, in this large cohort, another associated cause existed in all these stroke patients with MPN. 1 In the emergency setting of a stroke, diagnosing underlying MPN can be difficult. First, MPN patients share common cardiovascular comorbidities that affects stroke risk in the same way than it does in general population.…”

“…After stroke, 10/21 (48%) received antiaggregant therapy, nine received anticoagulation therapy (43%) and one was treated by both. Median initial NIHSS and NIHSS at discharge were four [1][2][3][4][5][6][7][8][9][10] and two [1][2][3][4] respectively. Three patients experienced a stroke recurrence (two had a history of stroke, and one patient had a stroke recurrence 2 months after the recorded event), and one (5%) patient died from stroke.…”