Lenalidomide-Induced Ischemic Cerebrovascular Disease in Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Gammopathy, and Skin Changes Syndrome

Mitsutake, Akihiko; Matsumoto, Hideyuki; Hatano, Keiko; Irie, Koreaki; Tsukada, Nobuhiro; Hashida, Hideji

doi:10.1016/j.jstrokecerebrovasdis.2017.12.039

Cited by 15 publications

(21 citation statements)

References 5 publications

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“…With respect to preventing recurrence, our study showed that IS recurred in 53.8% of patients with POEMS syndrome even after anti-thrombotic treatment, indicating that anti-thrombotic therapy is insufficient for POEMS patients with IS, which has been reported by others. 4,22,34,35 Previous reports 4,22,[45][46][47][48] have suggested that treatment of POEMS syndrome might be necessary to reduce the A B C risk of IS relapse, and our cohort also showed that none of the patients with POEMS had IS recurrence after successful anti-plasma cell treatment. Therefore, disease control of POEMS syndrome is more important than anti-thrombotic therapy to prevent IS recurrence.…”

Section: Discussionsupporting

confidence: 60%

Ischemic stroke in patients with POEMS syndrome

Feng¹,

Gao²,

Zhao³

et al. 2020

Blood Advances

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Polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, and skin changes (POEMS) syndrome is associated with increased risk for ischemic stroke (IS). Because POEMS syndrome is rare, little is known regarding the underlying mechanism and prognosis for IS in patients in whom it occurs. The medical records of patients with POEMS syndrome were screened between January 2018 and January 2000 at Peking Union Medical College Hospital to identify those with IS. The baseline characteristics, IS features, and patient outcomes were analyzed. Forty-one (8.0%) of 510 POEMS patients were documented to have IS. Patients with IS were older, had a higher percentage of Overall Neuropathy Limitation Scale score >4, and had a higher level of fibrinogen compared with those who did not have IS. Ninety-three percent of IS events occurred before or within 3 months after a diagnosis of POEMS. Of 41 occurrences of IS, 29 (46.3%) were multifocal. Recurrent IS was observed in 13 (31.7%) of 41 patients, but not in patients with successful anti-plasma cell therapy. The 3-year overall survival rate in patients with IS was 71.0% and for those without IS, it was 88.5% (P = .002). We showed that 8.0% of POEMS patients had IS, and most IS events occurred in POEMS patients not being treated effectively. Having IS was a predictor of unfavorable prognosis. Early diagnosis, immediate initiation of treatment for POEMS, and control of POEMS syndrome is key to reducing the occurrence of IS, improving survival, and preventing recurrence of IS.

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Section: Discussionsupporting

confidence: 60%

Ischemic stroke in patients with POEMS syndrome

Feng¹,

Gao²,

Zhao³

et al. 2020

Blood Advances

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“…The most common ocular symptoms reported were blurred vision in 15, diplopia in 5, and ocular pain in 3. In a series of 41 patients from China, there was a positive correlation between serum VEGF levels and the binocular mean retinal nerve fiber layer thickness . In another series of 94 patients, papilledema, which was found in approximately 50% of patients, was an adverse prognostic feature for overall survival …”

Section: Diagnosismentioning

confidence: 97%

“…The use of low molecular weight heparin or warfarin should be balanced against fall risk. Some authors recommend baseline head magnetic resonance angiography prior to starting lenalidomide and dexamethasone due to their experience with lenalidomide‐induced ischemic cerebrovascular disease in a patient with POEMS syndrome …”

Section: Management Of Poems Syndrome With Disseminated Bone Marrow Imentioning

confidence: 99%

POEMS Syndrome: 2019 Update on diagnosis, risk‐stratification, and management

2019

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Disease Overview: Polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes (POEMS) syndrome is a paraneoplastic syndrome due to an underlying plasma cell neoplasm. The major criteria for the syndrome are polyradiculoneuropathy, clonal plasma cell disorder (PCD), sclerotic bone lesions, elevated vascular endothelial growth factor, and the presence of Castleman disease. Minor features include organomegaly, endocrinopathy, characteristic skin changes, papilledema, extravascular volume overload, and thrombocytosis. Diagnoses are often delayed because the syndrome is rare and can be mistaken for other neurologic disorders, most commonly chronic inflammatory demyelinating polyradiculoneuropathy. POEMS syndrome should be distinguished from the Castleman disease variant of POEMS syndrome, which has no clonal PCD and typically little to no peripheral neuropathy but has several of the minor diagnostic criteria for POEMS syndrome. Diagnosis: The diagnosis of POEMS syndrome is made with three of the major criteria, two of which must include polyradiculoneuropathy and clonal PCD, and at least one of the minor criteria. Risk Stratification: Because the pathogenesis of the syndrome is not well understood, risk stratification is limited to clinical phenotype rather than specific molecular markers. Risk factors include low serum albumin, age, pleural effusion, pulmonary hypertension, and reduced eGFR. Risk-Adapted Therapy: For those patients with a dominant sclerotic plasmacytoma, first line therapy is irradiation. Patients with diffuse sclerotic lesions or disseminated bone marrow involvement and for those who have progression of their disease 3 to 6 months after completing radiation therapy should receive systemic therapy. Corticosteroids are temporizing, but alkylators are the mainstay of treatment, either in the form of low dose conventional therapy or high dose with stem cell transplantation. Lenalidomide shows promise with manageable toxicity. Thalidomide and bortezomib also have activity, but their benefit needs to be weighed against their risk of exacerbating the peripheral neuropathy. Prompt recognition and institution of both supportive care measures and therapy directed against the plasma cell result in the best outcomes.

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“…More cases have been reported regarding limb and coronary artery involvement, but cerebrovascular involvement has been rarely reported [6]. Cerebral infarction has been noted in only two cases who were diagnosed with POEMS syndrome and were on lenalidomide-dexamethasone combination therapy [8].…”

Section: Discussionmentioning

confidence: 99%

Ischemic Stroke in a Patient With Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Protein, and Skin Changes Syndrome Treated With Lenalidomide

Sodavarapu

Mahajan

2020

Cureus

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Polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes (POEMS) syndrome is an uncommon multisystemic disease associated with plasma cell dyscrasia. Due to the disease's rarity and an even rarer presentation of stroke in afflicted patients, a direct association between POEMS syndrome and stroke remains ambiguous. Thrombocytosis, hyperfibrinogenemia, and increased levels of inflammatory cytokines occur in this disease, which can predispose patients to thromboembolic events. Immunomodulators can also enhance thrombosis, the chances of which increase when they are combined with dexamethasone. We present a case of a 28-year-old patient with an ischemic stroke, which may have been triggered by the combination of POEMS syndrome-associated vasculitis and the thrombogenic nature of lenalidomide-dexamethasone therapy.

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Lenalidomide-Induced Ischemic Cerebrovascular Disease in Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Gammopathy, and Skin Changes Syndrome

Cited by 15 publications

References 5 publications

Ischemic stroke in patients with POEMS syndrome

Ischemic stroke in patients with POEMS syndrome

POEMS Syndrome: 2019 Update on diagnosis, risk‐stratification, and management

Ischemic Stroke in a Patient With Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Protein, and Skin Changes Syndrome Treated With Lenalidomide

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