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Published on: September 25, 2019
Cerebral infarction in POEMS syndrome: incidence, risk factors, and imaging characteristics
S A Dupont1, A Dispenzieri, M L Mauermann
1Department of Neurology, Mayo Clinic, Rochester, MN 55905, USA. dupont.stefan@mayo.edu
Insights
Patients with POEMS syndrome face a significant risk of cerebral infarction, particularly those with elevated platelet counts and bone marrow plasmacytosis. Aggressive management of stroke risk factors and POEMS syndrome treatment are crucial.
Area of Science:
- Neurology
- Hematology
- Oncology
Background:
- POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) is a rare multisystem disorder.
- Cerebral infarction is a serious complication, but its risk factors and incidence in POEMS syndrome are not well-established.
Purpose of the Study:
- To determine the incidence and risk factors of cerebral infarction in patients diagnosed with POEMS syndrome.
- To identify predictors of stroke in this specific patient population.
Main Methods:
- Retrospective observational study utilizing the Mayo Clinic dysproteinemia database.
- Identification of patients with POEMS syndrome and subsequent cerebral infarction after symptom onset.
- Evaluation of potential stroke predictors, including hematologic abnormalities and traditional risk factors.
Main Results:
- Cerebral infarction occurred in 10% of POEMS syndrome patients, with a 5-year risk of 13.4%.
- Hematologic abnormalities, specifically elevated platelet count and bone marrow plasmacytosis, were associated with increased stroke risk.
- Traditional stroke risk factors were not significantly different between stroke and non-stroke subgroups.
- Infarct etiologies included vascular abnormalities (dissection, stenosis) and embolism.
Conclusions:
- Plasma cell proliferation and thrombocytosis are key risk factors for cerebral infarction in POEMS syndrome.
- Aggressive management of modifiable stroke risk factors is recommended.
- Treatment of thrombocytosis and the underlying POEMS syndrome may reduce the risk of cerebral infarction.
Objectives:
To determine the risk factors and incidence of cerebral infarction associated with POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) syndrome.
Methods:
The Mayo Clinic dysproteinemia database was queried to identify patients with coded diagnosis of POEMS syndrome. Patients with cerebral infarction, occurring after the onset of POEMS-related symptoms, were selected. A retrospective observational study design was used to evaluate potential predictors of stroke in patients with POEMS syndrome.
Results:
A total of 9 patients (10%; 95% confidence interval 5.4-17.9) with cerebral infarction were identified (2 women, 22%). Traditional stroke risk factors were not significantly different between the stroke and nonstroke subgroups, but hematologic abnormalities such as elevated platelet count and bone marrow plasmacytosis differed between the 2 groups. Cerebral infarction occurrence after successful treatment of the underlying condition was not observed. CT and MRI data demonstrated a wide spectrum of infarct topography in these patients. Common stroke etiologies comprised suspected vascular structural abnormalities leading to vessel dissection and stenosis, in addition to embolism from a proximal source.
Conclusions:
The 5-year risk of cerebral infarction in patients with POEMS syndrome is 13.4%. Evidence of plasma cell proliferation within the bone marrow and elevated serum platelet count led to increased risk of cerebral infarction in this population. We conclude that known modifiable stroke risk factors should be aggressively managed. Treatment of thrombocytosis should be considered in patients without a contraindication. Treatment of the syndrome may be the best approach to decreasing risk of cerebral infarction in these patients.
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