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Managing Cerebrovascular Complications in Patients With MPN


Complications of note in patients with myeloproliferative neoplasm (MPNs) include ischemic stroke, intracerebral and subarachnoid hemorrhage, microbleeds, posterior reversible encephalopathy syndrome, and dural sinus and cerebral vein thrombosis.

With patients with hematological diseases often experiencing cerebrovascular complications, researchers have published a review of current management practices for these complications, including in rare hematologic malignancies like myeloproliferative neoplasm (MPNs).

Complications of note include ischemic stroke, intracerebral and subarachnoid hemorrhage, microbleeds, posterior reversible encephalopathy syndrome, and dural sinus and cerebral vein thrombosis. Strokes in particular pose a dual emergency for patients, explained the authors of the review, as they need both immediate treatment for the even and for their underlying hematologic disease.

“Hematological diseases are a rare cause of stroke, but cerebrovascular complications are frequent in patients with hematological diseases,” explained the researchers. “Cerebrovascular disease has a negative impact on their prognosis. The complex management of stroke in the context of the different hematological diseases requires close cooperation between hematologists, neurologists, neuroradiologists, and sometimes neurosurgeons.”

Thrombotic complications have been documented as a frequent complication in MPNs, including in one study that showed these patients have a 1.3- to 3.7-fold increased hazard ratio for developing vascular disease at 5 years compared with the general population. The 5-year risk of vascular disease ranged from 0.5% to 7.7% among patients with MPN.

Other research has suggested that stroke may be the first sign of an underlying MPN. A sample of over 500,000 hospitalizations among patients with essential thrombocytopenia (ET) found that over 20,000 were because of a stroke.

When it comes to managing the risk of cardiovascular disease, current treatment algorithms for MPNs like polycythemia vera and ET include recommendations for carefully assessing cardiovascular risk factors and use of low-dose aspirin.

“The risk of thrombotic events modulates the need for cytoreductive therapy, in addition to aspirin and phlebotomies, explained the researchers, adding, “Other risk factors in high-risk PV patients include other vascular risk factors for ischemic stroke or CVT, and JAK2 mutations. Hydroxyurea is the most commonly used cytoreductive drug. Busulfan is an alternative in older patients, while younger patients may receive interferon α and/or anagrelide. Recently, effective and nongenotoxic JAK inhibitors (ruxolitinib and fedratinib) have been developed and may be used in patients refractory to first-line therapies.”

Recommendations are similar for patients with ET, and cytoreduction to lower platelet counts is advised in some patients with intermediate and all patients with high-risk disease, including those who have previously had a stroke.

In patients with either PV or ET who do experience stroke, it’s recommended that platelet counts are frequently monitored during unfractionated heparin treatment in order to detect heparin-induced thrombocytopenia.


Ferro J, Infante J. Cerebrovascular manifestations in hematological diseases: an update. J Neurol. 2021;268(9):3480-3492.

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