Ischemic stroke in patients with POEMS syndrome

Feng, Jun; Gao, Xuemin; Zhao, Hao; He, Tianbiao; Zhang, Congli; Shen, Kaini; Zhang, Lu; Cao, Xinxin; Qian, Ming; Zhou, Daobin; Li, Jian

doi:10.1182/bloodadvances.2020001865

Cited by 11 publications

(16 citation statements)

References 44 publications

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“…Ischemic stroke occurred in 7% of patients, compared with published rates of 8% and 9% 6,8 . Consistent with previous reports, most thromboses occurred prior to initiation of therapy, arterial events were more common than venous events, and several patients developed thrombosis despite anticoagulation 6–8 . We confirm that high platelet count is a risk factor for arterial thrombosis, 5,6 but identify new risk factors for arterial thrombosis, including elevated hemoglobin/hematocrit (especially in men), splenomegaly, and extravascular volume overload.…”

Section: Discussionsupporting

confidence: 88%

“…We report a thrombosis rate of 27% in patients with POEMS, approximating previous estimates of 20% and 30% 7,9 . Ischemic stroke occurred in 7% of patients, compared with published rates of 8% and 9% 6,8 . Consistent with previous reports, most thromboses occurred prior to initiation of therapy, arterial events were more common than venous events, and several patients developed thrombosis despite anticoagulation 6–8 .…”

Section: Discussionsupporting

confidence: 87%

“…6 However, more recent investigations found no association between thrombocytosis and either ischemic stroke or thrombosis in general. 7,8 In one study, thrombotic risk was highest during the active disease phase, defined as a serum VEGF > 1000 pg/ml, but no additional risk factors were identified. 7 The aims of this study are to provide a comprehensive overview of thrombosis in POEMS syndrome and to ascertain risk factors for POEMS-associated thrombosis.…”

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confidence: 99%

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Characteristics and risk factors for thrombosis in POEMS syndrome: A retrospective evaluation of 230 patients

Mellors

Kourelis

et al. 2021

American J Hematol

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Thromboses are prevalent in POEMS syndrome, but few risk factors for POEMSassociated thrombosis have been identified. The objective of this study is to identify novel risk factors for POEMS-associated thrombosis. In this retrospective cohort of 230 POEMS patients, 27% developed thrombosis. Arterial events were slightly more common than venous. Stroke accounted for 26% of all thromboses and 53% of arterial events. There were differences in baseline features between the thrombosis group and the no thrombosis group, and these were driven by patients with arterial thrombosis. Risk factors for arterial thrombosis included thrombocytosis, elevated hemoglobin/hematocrit, extravascular volume overload, and splenomegaly. Hyperprolactinemia appeared to be a risk factor for venous thrombosis. The risk of thrombosis was most striking among men with elevated hemoglobin (32% vs. 5%, p < .001) and hematocrit (42% vs. 5%, p < .001) compared to men without. Most thromboses occurred prior to POEMS directed therapy, and most that occurred during therapy happened within 3 months of diagnosis. Twenty-one percent of patients with thrombosis had recurrence. In recognition of high overall rates of thrombosis in this population, all patients with POEMS syndrome should receive prophylactic antiplatelet therapy, and clinicians should consider anticoagulation in patients with risk factors for POEMS-associated thrombosis. 1 | INTRODUCTION POEMS syndrome is a rare, multisystem paraneoplastic disorder driven by an aberrant plasma cell clone. The POEMS acronym, coined by Bardwick in 1980, summarizes five common associated signs and symptoms of this disease (polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes). 1 Additional features include papilledema, extravascular volume overload, sclerotic bone lesions, thrombocytosis, elevations in vascular endothelial growth factor (VEGF), and Castleman disease.Although not included in formal diagnostic criteria, the association between POEMS syndrome and thrombosis is well established in case reports and larger observational studies. [2][3][4][5][6][7][8] Thrombosis occurs in 20% to 30% of POEMS patients, and ischemic stroke in 8% to 10%. [5][6][7][8][9] Studies on risk factors for thrombosis in POEMS syndrome, however, are limited and feature conflicting results. In one report, bone

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Section: Discussionsupporting

confidence: 88%

Section: Discussionsupporting

confidence: 87%

mentioning

confidence: 99%

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Characteristics and risk factors for thrombosis in POEMS syndrome: A retrospective evaluation of 230 patients

Mellors

Kourelis

et al. 2021

American J Hematol

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“…Ischemic stroke is a well-known complication in patients with POEMS. Three recent case series confirmed the typical features of stroke associated with POEMS [ 43 – 45 ]. The frequency of ischemic stroke among patients with POEMS was 8%.…”

Section: Polyneuropathy Organomegaly Endocrinopathy Monoclonal Protein and Skin Changes Syndrome (Poems)mentioning

confidence: 67%

Cerebrovascular manifestations in hematological diseases: an update

Ferro

Infante

2021

J Neurol

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Patients with hematological diseases often experience cerebrovascular complications including ischemic stroke, intracerebral and subarachnoid hemorrhage, microbleeds, posterior reversible encephalopathy syndrome, and dural sinus and cerebral vein thrombosis (CVT). In this update, we will review recent advances in the management of cerebrovascular diseases in the context of myeloproliferative neoplasms, leukemias, lymphomas, multiple myeloma, POEMS, paroxysmal nocturnal hemoglobinuria (PNH), thrombotic thrombocytopenic purpura (TTP), and sickle-cell disease. In acute ischemic stroke associated with hematological diseases, thrombectomy can in general be applied if there is a large vessel occlusion. Intravenous thrombolysis can be used in myeloproliferative neoplasms and sickle-cell anemia, but in other diseases, a case-by-case evaluation of the bleeding risks is mandatory. Patients with sickle-cell disease and acute stroke need very often to be transfused. In PNH, acute ischemic stroke patients must be anticoagulated. Most patients with CVT can be treated with low-molecular weight heparin (LMWH) acutely, even those with leukemias. Prevention of recurrence of cerebral thrombotic events depends on the control of the underlying disease, combined in some conditions with antithrombotic drugs. The recent introduction of specific monoclonal antibodies in the treatment of PHN and TTP has dramatically reduced the risk of arterial and venous thrombosis.

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“…[22][23][24] Cohort studies from the Mayo Clinic and Peking Union Medical College Hospital have addressed this issue in detail. 25,26 Conversely, VEGF can promote the growth of new blood vessels and capillaries around ischemic blood vessels to improve the blood supply. 27 Meanwhile, POEMS syndrome is a plasma cell disease.…”

Section: Discussionmentioning

confidence: 99%

Missed diagnosis of POEMS syndrome with onset of progressive fatigue and numbness: a case report

et al. 2021

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POEMS syndrome is a rare multisystem disease associated with an underlying plasma cell disorder. Its name is an acronym for peripheral neuropathy (P), endocrinopathy (E), organomegaly (O), monoclonal plasma cell proliferative disorder (M), and skin changes (S). This case report describes a patient with POEMS syndrome who presented with progressive fatigue and numbness in the lower extremities. Initially, the patient was erroneously diagnosed with diabetes and diabetic peripheral neuropathy because of the endocrinopathy associated with POEMS syndrome. After a second hospitalization, the patient was diagnosed with POEMS syndrome and recovered with alkylator therapy and a peripheral blood stem cell transplant. The patient’s overall condition was improved at the 1-year follow-up. POEMS syndrome should be considered if a patient presents with endocrinopathy and unexplained peripheral neuropathy.

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Ischemic stroke in patients with POEMS syndrome

Cited by 11 publications

References 44 publications

Characteristics and risk factors for thrombosis in POEMS syndrome: A retrospective evaluation of 230 patients

Characteristics and risk factors for thrombosis in POEMS syndrome: A retrospective evaluation of 230 patients

Cerebrovascular manifestations in hematological diseases: an update

Missed diagnosis of POEMS syndrome with onset of progressive fatigue and numbness: a case report

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