Papilloedema is an independent prognostic factor for POEMS syndrome

Cui, Rong-Tai; Yu, ShengYuan; Huang, Xusheng; Zhang, Jiatang; Tian, Chenglin; Chen, Pu

doi:10.1007/s00415-013-7143-4

Cited by 40 publications

(41 citation statements)

References 23 publications

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“…ODO was detected bilaterally in 13 patients (76%), which is consistent with earlier reports that bilateral ODO was the most common (30%–70%) sign in POEMS syndrome 1 4 6 8 11. The cause of the ODO in POEMS syndrome has not been determined, but it has been suggested that an elevated ICP,7–9 11 vasculitis,18 presence of cerebrospinal fluid proteins,11 infiltrations into the optic nerve,19 or increased VEGF levels4 8 10 20 may be involved.…”

Section: Discussionsupporting

confidence: 91%

Correlation between peripapillary retinal thickness and serum level of vascular endothelial growth factor in patients with POEMS syndrome

et al. 2015

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Section: Discussionsupporting

confidence: 91%

Correlation between peripapillary retinal thickness and serum level of vascular endothelial growth factor in patients with POEMS syndrome

et al. 2015

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“…Therefore, it is unsurprising that most previous studies concentrated on the survival value of a single clinical variable [5,6,24,25]. A critical question always asked by physicians caring 9 for such patients is the risk factors that could predict overall survival independently.…”

Section: Discussionmentioning

confidence: 96%

Prognostic study for overall survival in patients with newly diagnosed POEMS syndrome

Wang

et al. 2016

Leukemia

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POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes) is a multisystem disorder with a good long-term prognosis. In its dozens of clinical features, those with independent prognostic value are still not well characterized. We retrospectively included 362 patients with newly diagnosed POEMS syndrome at our institute from 2000 to 2015. On the basis of a randomized sample splitting, we first identified four baseline clinical variables, including age >50 years (hazards ratio (HR) 4.07, 95% confidence interval (CI) 1.41-11.76, P=0.009), pulmonary hypertension (HR 3.99, 95% CI 1.44-11.04, P=0.008), pleural effusion (HR 3.81, 95% CI 1.23-11.79, P=0.02) and estimated glomerular filtration rate <30 ml/min/1.73 m (HR 8.25, 95% CI 2.18-31.25, P=0.002), associated with inferior overall survival in the derivation cohort, with the use of multivariate Cox regression model. These factors were incorporated together to develop a prognostic nomogram. Concordance index calculation (0.727, 95% CI 0.601-0.853, P=0.018) and calibration curve plotting demonstrated its significant predictive and discriminatory capacity in the validation cohort. This nomogram could be a useful and convenient tool in clinical practice to evaluate individualized prognosis in patients with newly diagnosed POEMS syndrome.

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“…The most common ocular symptoms reported were blurred vision in 15, diplopia in 5, and ocular pain in 3. In another series of 94 patients, papilledema, which was found in 50% of patients, was an adverse prognostic feature for overall survival [53].…”

Section: Diagnosismentioning

confidence: 89%

“…Whether the digital clubbing seen in POEMS is a reflection of underlying pulmonary hypertension and/or parenchymal disease is yet to be determined. Impaired DLCO has been shown to be an adverse prognostic factor in another series [53]. The histologic findings of the dermis have been reported to range from non-specific to glomeruloid hemangiomata to vascular abnormalities in apparently normal dermis [81][82][83].…”

Section: Diagnosismentioning

confidence: 99%

POEMS syndrome: Update on diagnosis, risk‐stratification, and management

2015

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Disease overview: 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 plasma cell disorder, 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. The number of clinical criteria is not prognostic, but the extent of the plasma cell disorder is. Those patients with an iliac crest bone marrow biopsy that does not reveal a plasma cell clone are candidates for local radiation therapy; those with a more extensive or disseminated clone will be candidates for systemic therapy.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 3–6 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. The benefit of anti‐VEGF antibodies is conflicting. Prompt recognition and institution of both supportive care measures and therapy directed against the plasma cell result in the best outcomes. Am. J. Hematol. 90:951–962, 2015. © 2015 Wiley Periodicals, Inc.

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Papilloedema is an independent prognostic factor for POEMS syndrome

Cited by 40 publications

References 23 publications

Correlation between peripapillary retinal thickness and serum level of vascular endothelial growth factor in patients with POEMS syndrome

Correlation between peripapillary retinal thickness and serum level of vascular endothelial growth factor in patients with POEMS syndrome

Prognostic study for overall survival in patients with newly diagnosed POEMS syndrome

POEMS syndrome: Update on diagnosis, risk‐stratification, and management

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