Polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, skin changes (POEMS) syndrome refractory to complete serum vascular endothelial growth factor (VEGF) blockade: insights from sequential VEGF monitoring

Guitard, Joëlle; Pillet, Adèle; Soubrier, Martin; Modesto, Anne; Chauveau, Dominique

doi:10.1093/ndtplus/sfp094

Cited by 3 publications

(3 citation statements)

References 11 publications

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“…Patients with POEMS syndrome tend to have a higher skin vessel density than healthy individuals, but the density itself is not related to VEGF levels (22). The anti-VEGF antibody bevacizumab has been tested for the treatment of POEMS syndrome, but the results were ambiguous (23)(24)(25)(26)(27)(28)(29)(30)(31)(32). Skin responses to bevacizumab therapy can be good or remain stable in different reports.…”

Section: Discussionmentioning

confidence: 99%

Skin Responses in Newly Diagnosed Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Gammopathy, and Skin Changes (POEMS) Syndrome After Therapy With Low-Dose Lenalidomide Plus Dexamethasone

et al. 2021

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BackgroundPolyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome is a rare paraneoplastic disease with a high prevalence of skin symptoms. Few studies have focused on skin responses to systemic treatment of this disease.ObjectiveTo evaluate skin responses after treatment with low-dose lenalidomide plus dexamethasone and determine their relationship with vascular endothelial growth factor (VEGF) and hematological responses.MethodsA total of 41 consecutive Chinese patients who were at least 18 years of age with newly diagnosed POEMS syndrome were enrolled. 36 of them completed 12 cycles of treatment. Skin, serum VEGF, hematological and neurological responses were documented at 1, 2, 3, 6, 9, and 12 months during treatment.ResultsSkin manifestations were not associated with serum VEGF levels at baseline. Of the patients with hyperpigmentation, hemangiomas, hypertrichosis, white nails, or acrocyanosis separately, 26/34 (76.5%), 11/30 (36.7%), 14/15 (93.3%), 21/21 (100%), and 4/4 (100%) achieved complete responses at 12 months. Reduction in hyperpigmentation after 12 months reflected a good VEGF response (P = 0.017).ConclusionLow-dose lenalidomide plus dexamethasone therapy is effective in reversing skin changes in patients with POEMS syndrome.Clinical Trial RegistrationClinicaltrials.gov, identifier NCT01816620

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Section: Discussionmentioning

confidence: 99%

Skin Responses in Newly Diagnosed Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Gammopathy, and Skin Changes (POEMS) Syndrome After Therapy With Low-Dose Lenalidomide Plus Dexamethasone

et al. 2021

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“…Some case reports have shown misleading results with bevacizumab and worsening of disease leading to death. [ 16 , 17 ] However, other case studies have shown a benefit, though these have been limited by short or unclear follow up periods, and bevacizumab has been used following, or in addition to, other treatments including cyclophosphamide with dexamethasone. [ 18 , 19 ] At present, high-dose chemotherapy-conditioned autologous stem cell transplantation is the gold standard treatment for POEMS syndrome, showing good hematological control, neurological response, and good survival rates.…”

Section: Discussionmentioning

confidence: 99%

Atypical polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes syndrome without polyneuropathy

Li¹,

Duan³

et al. 2020

Medicine

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Introduction: Polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes (POEMS) syndrome is a rare paraneoplastic syndrome that occurs secondary to an underlying plasma cell disorder. The diagnosis of POEMS syndrome is 2 of the mandatory major criteria (polyneuropathy and monoclonal plasma cell disorder), 1 of the other major criteria (Castleman's disease, sclerotic bone lesions, or vascular endothelial growth factor elevation), and at least 1 of the minor criteria (organomegaly, extravascular volume overload, endocrinopathy, skin changes, papilledema, thrombocytosis, or polycythemia). However, some cases do not fully meet the diagnostic criteria, such cases are referred to as incomplete or atypical POEMS syndrome. Patient concerns: A 58-year-old Chinese female was admitted to our department of neurology with weakness of both arms and legs. In addition,it's found that she had skin manifestations, lymphadenopathies, pedal edema, immunoglobin - A-λ restricted paraproteinemia, and elevated vascular endothelial growth factor and other features , b ut without polyneuropathy. Diagnoses: we made a diagnosis of atypical POEMS syndrome without polyneuropathy. Interventions: Two drug regimens were recommended: VAD (Vincristine, Adriamycin, Dexamethasone) and bortezomib. Finally, the VAD strategy was performed. Outcomes: The patient's limb strength and pain improved and enzyme parameters decreased gradually after 4 weeks. However, the treatment was still not perfect. Conclusion: We reported a rare case of POEMS syndrome without polyneuropathy. We hope similar cases will be reported in the future.

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“…A number of small studies and case reports have shown mixed results with bevacizumab, including treatment failure (except VEGF response), and worsening of disease leading to death [67–70]. Some case studies have shown benefit, though these have been limited by short or unclear follow-up periods, and bevacizumab has been used following, or in addition to other treatments including cyclophosphamide with dexamethasone [68, 71].…”

Section: Managementmentioning

confidence: 99%

POEMS syndrome: clinical update

Brown

Ginsberg

2018

J Neurol

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POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes) is a rare paraneoplastic syndrome, caused by a plasma cell proliferative disorder, which is most commonly lambda restricted. The neurological hallmark, which forms one of the mandatory criteria for diagnosis, is a subacute onset demyelinating neuropathy, which can be rapidly disabling and painful. A number of multi-system features are also characteristic of this disorder, and certainly not restricted to those included in its acronym, which though limited, remains a useful and memorable name, helping distinguish POEMS syndrome from other paraproteinaemic neuropathies. The discovery of vascular endothelial growth factor (VEGF) in association with POEMS syndrome has been extremely useful in aiding clinical diagnosis, and monitoring response to treatment, as well as helping understand the underlying mechanism of disease. Interestingly, however, treatment targeting VEGF has been disappointing, suggesting other disease mechanisms or inflammatory processes are also important. Current understanding of the pathogenesis of POEMS syndrome is outlined in detail in the accompanying article by Cerri et al. Here, we review the clinical features of POEMS syndrome, differential diagnosis and available treatment options, based on current literature.

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Polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, skin changes (POEMS) syndrome refractory to complete serum vascular endothelial growth factor (VEGF) blockade: insights from sequential VEGF monitoring

Cited by 3 publications

References 11 publications

Skin Responses in Newly Diagnosed Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Gammopathy, and Skin Changes (POEMS) Syndrome After Therapy With Low-Dose Lenalidomide Plus Dexamethasone

Skin Responses in Newly Diagnosed Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal Gammopathy, and Skin Changes (POEMS) Syndrome After Therapy With Low-Dose Lenalidomide Plus Dexamethasone

Atypical polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes syndrome without polyneuropathy

POEMS syndrome: clinical update

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