Update on the POEMS syndrome

Kim, Yu Ri

doi:10.5045/br.2022.2022001

Cited by 10 publications

(5 citation statements)

References 40 publications

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“…Changes in treatment strategies from steroid-based therapies to plasma cell-targeting treatments and the introduction of ASCT have enhanced the overall prognosis of POEMS syndrome. 25 In our study, recent diagnosis year > 2014 was associated with a trend for longer PFS in all treated patient groups and also in the ASCT group. Before December 2013, 11 patients (42.3%) were treated with conventional chemotherapies and 2 (7.7%) were treated with thalidomide.…”

Section: Discussionsupporting

confidence: 51%

A Multicenter Analysis of Clinical Features and Long-Term Outcomes of POEMS Syndrome in Korea

Kook,

Jang,

Min

et al. 2024

J Korean Med Sci

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Background POEMS syndrome is a rare form of plasma cell dyscrasia characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal proteins, and skin changes. Owing to its low incidence, there are few reports regarding this syndrome. This multicenter study included 84 patients diagnosed with POEMS syndrome in South Korea. Methods We retrospectively evaluated 84 patients diagnosed with POEMS syndrome at 8 hospitals in South Korea between January 2000 and October 2022. The clinical characteristics and treatment outcomes were analyzed. Results The median patient age was 53 years (range, 26–77 years), and 63.1% of the patients were male. All patients had peripheral neuropathy, and 81 (96.4%) had monoclonal plasma cell proliferation. Plasma vascular endothelial growth factor levels were available for 32 patients with a median of 821 pg/mL (range, 26–12,900 pg/mL). Other common features included skin changes (54.2%), volume overload (71.4%), and organomegaly (72.6%). Of the 84 patients, 75 received initial treatment (local radiotherapy, 6 [8.0%]; chemotherapy, 17 [22.7%]; both chemotherapy and local radiotherapy, 9 [12.0%]), upfront autologous stem cell transplantation (ASCT), 43 (57.3%; with induction chemotherapy, n = 12, 16.0%; without induction chemotherapy, n = 31, 41.3%). The median follow-up duration was 40.7 months. The 5-year overall survival (OS) was 78%, and the 5-year progression-free survival (PFS) was 55%. Patients who underwent upfront ASCT and were diagnosed after 2014 had a longer OS and PFS. Conclusion The demographics of Korean patients with POEMS syndrome were similar to those reported previously. Because of the introduction of new treatment agents and the reduced rate of transplant-related mortality related to ASCT, the treatment outcomes of Korean patients with POEMS syndrome have improved in recent years.

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Section: Discussionsupporting

confidence: 51%

A Multicenter Analysis of Clinical Features and Long-Term Outcomes of POEMS Syndrome in Korea

Kook,

Jang,

Min

et al. 2024

J Korean Med Sci

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“…The above symptoms may be accompanied by osteosclerotic fractures, oedema, pleural fluid, thrombocytosis, polycythaemia and Castelman’s disease. In 90% of patients, skin lesions are present, including thickening of the skin - mainly of the hands and feet ( 19 , 20 ); sclerodactyly (16% of cases) ( 12 ), hyperpigmentation, telangiectasias, excessive hairiness and glomus haemangiomas ( 19 , 20 ). Raynaud’s phenomenon is observed in 20% of patients ( 12 ).…”

Section: Inflammatory Diseasesmentioning

confidence: 99%

“…The skin lesions are accompanied by abnormalities in the examination of respiratory mechanics. The prevalence of the disease in the population is estimated to be 0.003%, more commonly in men ( 19 , 20 ) aged 60-70 years ( 12 ).…”

Section: Inflammatory Diseasesmentioning

confidence: 99%

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Scleroderma and scleroderma-like syndromes

Romanowska-Próchnicka,

Dziewit,

Lesiak

et al. 2024

Front. Immunol.

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Systemic sclerosis is a systemic connective tissue disease whose main pathophysiological mechanism is a progressive fibrosis of internal organs and skin leading to thickening and induration. Blood vessels may also be involved. However, systemic scleroderma is not the only disease causing cutaneous sclerosis. There is a group of diseases that mimic scleroderma in their clinical presentation - these are scleroderma-like syndromes. A distinction can be made between syndromes of inflammatory/autoimmune, genetic, metabolic, toxic, drug-induced, occupational, paraneoplastic and syndromes caused by deposition disorders. In the following paper, we have reviewed the literature on scleroderma-like syndromes. We have outlined the factors predisposing to the development of each disease, its pathogenesis, clinical presentation, diagnostic and treatment process and the differences between each syndrome and systemic scleroderma.

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“…The use of autologous stem cell transplantation (ASCT) following high-dose chemotherapy is a pillar of treatment of patients with various hematological malignancies [ 1 , 2 ]. Over the years, the use of this therapeutic option has been consolidated also in plasma cell disorders other than multiple myeloma (MM), solid tumors such as neuroblastoma or germinal tumors, and autoimmune diseases [ 3 , 4 , 5 , 6 , 7 ]. In candidates for ASCT, apheresis procedures should secure a minimum cell dose of 2 × 10 6 CD34+ cells/kg [ 8 , 9 ].…”

Section: Introductionmentioning

confidence: 99%

Day -1 CD34+ Cells and Platelet Count Predict the Number of Apheresis in Poor-Mobilizer Patients Rescued by Plerixafor

Valentini

Pellegrino

Putzulu

et al. 2023

JCM

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Plerixafor is widely used as up-front treatment with G-CSF to enhance peripheral blood hematopoietic stem cell output in patients failing previous mobilizations. Less frequently, plerixafor is used to rescue an unsatisfactory mobilization following chemotherapy (CT) and G-CSF. This study investigates if pre-collection factors affect the CD34+ cell harvest in chemotherapy and G-CSF mobilizations rescued by plerixafor. Clinical and hematological data relative to patients, mobilization, and apheresis products were retrospectively examined. The outcome was completing a target cell dose ≥ 2 × 106 CD34+ cells /Kg at first apheresis. The effect exerted on the outcome by patient- and disease-related factors was investigated by univariate and multivariate logistic regression analysis. The analysis included data from 42 patients affected by hematological (39 patients) and non-hematological malignancies (three patients). Twenty-nine patients (69%) attained the target cell dose at first apheresis. Twelve out of the remaining 13 patients received an additional plerixafor administration, and all accomplished the transplant dose at a second apheresis procedure. Day -1 CD34+ PB count (OR1.46, 95% CI 1.1–1.9, p = 0.008) and platelet count (OR1.0, 95% CI 1.0–1.0, p = 0.033) predicted the achievement of the target dose at first apheresis, independently of pre-mobilization CT, radiation therapy, and disease status at mobilization. At ROC curve analysis, the best cut-off value predicting the successful collection at first apheresis was 7.5/µL for Day -1 CD34+ cell count (AUC 0.830, 0.69 sensitivity, and 0.92 specificity) and 75 × 109/L for Day -1 platelet count (AUC = 0.736, 0.65 sensitivity and 0.85 specificity). In conclusion, on-demand plerixafor rescue allows a successful stem cell collection, irrespectively of disease type and status, prior CT lines, and radiation exposure. Pre-apheresis CD34+ cells and platelet count predict the need for one or two aphereses.

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Update on the POEMS syndrome

Cited by 10 publications

References 40 publications

A Multicenter Analysis of Clinical Features and Long-Term Outcomes of POEMS Syndrome in Korea

A Multicenter Analysis of Clinical Features and Long-Term Outcomes of POEMS Syndrome in Korea

Scleroderma and scleroderma-like syndromes

Day -1 CD34+ Cells and Platelet Count Predict the Number of Apheresis in Poor-Mobilizer Patients Rescued by Plerixafor

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