What is the best first-line treatment for POEMS syndrome: autologous transplantation, melphalan and dexamethasone, or lenalidomide and dexamethasone?

Zhao, Hao; Huang, Xiao Ping; Gao, Xuemin; Cai, Hao; Zhang, Lu; Feng, Jun; Cao, Xinxin; Zhou, Daobin; Li, Jian

doi:10.1038/s41375-019-0391-2

Cited by 42 publications

(60 citation statements)

References 21 publications

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“…Patients receiving lenalidomide and dexamethasone had the shortest PFS and a trend for a shorted OS. It should also be noted that those patients undergoing ASCT had the lowest risk disease . In a similar vein, Bianco and colleague in a much smaller series (n = 15) systematically compared changes seen among patients receiving ASCT or lenalidomide and dexamethasone found changes neurologic changes and 1 and 2 years were comparable for the two therapies …”

Section: Management Of Poems Syndrome With Disseminated Bone Marrow Imentioning

confidence: 86%

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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Section: Management Of Poems Syndrome With Disseminated Bone Marrow Imentioning

confidence: 86%

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

2019

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“…The long‐term outcome of melphalan plus dexamethasone in POEMS syndrome was reported by Zaho et al. Among 79 patients, the 3‐year progression‐free survival was 85%. However, melphalan is thought to be not preferable because of blood stem cell damage and secondary myelodysplastic syndrome, especially in long‐term management.…”

Section: Treatmentmentioning

confidence: 76%

Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes syndrome: Diagnosis, treatment and the current status in Japan

Suichi

Misawa

2020

Clinical & Exp Neuroim

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Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes (POEMS) syndrome is a rare cause of demyelinating polyneuropathy associated with monoclonal proliferation of plasma cells. Because the disorder shows diverse symptoms, its diagnosis is often difficult. Previously reported diagnostic criteria had many items, and their accuracy was not confirmed. Recently, new diagnostic criteria have been proposed, which consist of just seven items and have better diagnostic accuracy. Since around 2000, treatments for myeloma, such as high‐dose chemotherapy with autologous stem cell transplantation, immunomodulatory drugs and proteasome inhibitors, have been applied to polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes syndrome. A recent national survey carried out in Japan showed that the prognosis has been prominently improved by these plasma cell‐targeting therapeutic interventions: 10‐year overall survival was 93%. Conversely, 24% of the patients were still treated with corticosteroid alone as an initial treatment, suggesting that recognition of the disorder is not sufficient. Accurate diagnosis and proper treatment in the early phase of the disease are essential to further improve the prognosis of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes syndrome. The present review focuses on diagnosis, treatment and the current status of the disease in Japan.

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“…Current systematic therapies include high-dose chemotherapy with autologous stem cell transplantation (ASCT), alkylator-based therapy, and therapy with novel agents. All three treatments can achieve acceptable remission rates and survival [10][11][12] .…”

Section: Discussionmentioning

confidence: 99%

A retrospective analysis of endocirnopathies in 136 Chinese patients with POEMS syndrome

Shao¹,

Zhang²,

Xianling³

et al. 2020

Preprint

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Background: POEMS syndrome is a rare multisystem disease but with a wide spectrum of clinical endocrinopathies. The patients with POEMS syndromes may present with one or more hormone disorders during disease course, but such phenomenon has usually been underestimated. In this report, the prevalence and clinical characteristics of Endocrine abnormalities in a large Chinese cohort with POEMS syndromes were summarized. Methods: Retrospective review of patients with a definite diagnosis of POEMS syndrome in our hospital between January 2000 and January 2020 were performed. The clinical data about endocrine abnormalities were extracted from their medical records and analysis.Results: This study comprised 136 patients (95 male, 41 female) with a median age of 48(40-56) years old. The endocrine abnormalities were more common (127 cases, 93.38%) in patients with POEMS syndromes. The prevalence of single endocrinopathy and multiple endocrinopathies was 12.60% (16/127 cases) and 87.40% (111/127) respectively. The most frequent endocrinopathy was hypogonadism (98/136, 72.06%), followed by hypothyroidism (83/136, 61.03%), hypocalcemia (50/136, 36.76%), hyperprolactinemia (47/136, 34.56%),abnormal glucose metabolism (41/136, 30.15%) and adrenal insufficiency (41/136, 30.15%).In POEMS syndromes, nearly most of endocrine organs could be involved.In patients with multiple endocrinopathies, the percentage of 2, 3, 4, 5 and 6 kind of endocrine axes involved were 29.92%(38/127), 30.71%(39/127), 17.32%(22/127), 7.09%(9/127) and 2.36%(3/127), respectively. It results in complex clinical presentation of hormone disorders, including overt or sublinical situation.Conclusions: In Conclusion, this study provides an overview of the abnormalities observed in a large series in China. Endocrinopathy manifestations in POEMS syndromes are more frequent, and its clinical complicacy should be paid more attention to. To patients with definite diagnosis of POEMS syndromes, early and thorough endocrine evaluation should be performed.

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What is the best first-line treatment for POEMS syndrome: autologous transplantation, melphalan and dexamethasone, or lenalidomide and dexamethasone?

Cited by 42 publications

References 21 publications

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

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

Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes syndrome: Diagnosis, treatment and the current status in Japan

A retrospective analysis of endocirnopathies in 136 Chinese patients with POEMS syndrome

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