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

Gao, Yajuan; Zhang, Shiyu; Yang, Lu; Li, Jian; Liu, Yuehua; Wang, Tao

doi:10.3389/fimmu.2021.681360

Cited by 3 publications

(3 citation statements)

References 43 publications

(54 reference statements)

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“…Skin changes improve in most patients over months 61 and should be assessed regularly. Purple discoloration of the extremities may persist even after successful treatment and may not signify treatment failure.…”

Section: Assessing Response To Treatment and Monitoring Strategiesmentioning

confidence: 99%

Comprehensive Diagnosis and Management of POEMS Syndrome

et al. 2022

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Polyneuropathy Organomegaly, Endocrinopathy, Monoclonal protein and Skin changes syndrome is a rare multisystem condition with a range of manifestations which are often overlooked as trivial comorbidities, until their whole triggers the possibility of the diagnosis. The diagnosis is typically delayed by 12–16 months, by which time patients can be severely disabled. There are no established consensus guidelines. We provide clinicians a comprehensive blueprint for managing POEMS from diagnostic suspicion through the work-up, selection of therapy, follow-up, and treatment of relapse based on published evidence and our large single-center experience. A multidisciplinary approach is essential including expert hematologists, neurologists, histopathologists, radiologists, and neurophysiologists. The aim of treatment is to eradicate the underlying plasma cell dyscrasia, but there are limited trial data to guide treatment decisions. Supportive care considerations include management of endocrinopathy, neuropathy, thrombosis, and infection. Response assessment is centered on clinical, neuropathy, hematological, vascular endothelial growth factor, and radiological criteria. Future clinical trials are welcomed in this setting where evidence is limited.

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Section: Assessing Response To Treatment and Monitoring Strategiesmentioning

confidence: 99%

Comprehensive Diagnosis and Management of POEMS Syndrome

et al. 2022

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“…In POEMS syndrome, the primary aim of treatment is to target plasma cells and suppress the production of the toxic monoclonal protein. Autologous stem cell transplantation (ASCT) [5], melphalan [6], immunomodulatory agents(thalidomide/lenalidomide) [7,8] and bortezomib, [9] etc., had good therapeutic effects in clinical application. Treating POEMS syndrome with these therapies is particularly tempting, not only because of their excellent action against plasma cells, but also owing to reversion of renal impairment.…”

Section: Introductionmentioning

confidence: 99%

Efficacy of novel agents in patients with nephropathy associated with POEMS syndrome

et al. 2022

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Objective To evaluate the clinical characteristics and outcomes of patients with nephropathy associated with POEMS syndrome who received novel agents in combination with dexamethasone therapy, and renal pathological changes based on repeat biopsy in some patients after these novel-agent-based therapies. Methods The records of patients with nephropathy associated with POEMS syndrome in a single hospital from May 2017 to February 2021 were retrieved and studied in detail. All the patients received four cycles of initial novel-agent-based regimens such as bortezomib and dexamethasone (BD) or thalidomide plus dexamethasone (TD) or lenalidomide plus dexamethasone (RD) treatment. We further evaluated the pathological efficacy of these novel agents by repeat renal biopsy. Results Twelve patients with an average age of 48.6 ± 8.3 years diagnosed with nephropathy associated with POEMS syndrome were enrolled in this study. The duration from disease onset to renal biopsy was 28(8.3 ~ 54.5) months. All patients achieved good clinical responses in different degree after four cycles of initial novel agents in combination with dexamethasone therapy. After the treatment with novel-agent-based regimens, the levels of proteinuria decreased in most patients and were negative in five patients. The levels of serum creatinine (SCr) decreased in ten patients. Serum M protein was negative in four patients and still positive in the other eight patients. The levels of serum vascular endothelial growth factor (VEGF) were detected in seven patients, which were all decreased. The levels of interleukin-6 (IL-6) were detected in eight patients, which were also decreased. Repeat biopsies were performed after four cycles of novel-agent-based therapies in four patients who were all treated with BD treatment. Mesangiolysis, mesangial cells proliferation, endothelial cells proliferation, subendothelial space widening and acute renal tubulointerstitial lesions improved, the chronic renal tubulointerstitial lesions were stable. Conclusions Novel agents improved clinical manifestations in patients with nephropathy associated with POEMS syndrome. In addition, novel-agent-based regimens such as BD treatment improved renal pathological manifestations, which suggested that novel agents could improve renal prognosis of the patients from the perspective of renal pathology.

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“…Due to the rarity of this disease, randomized clinical trials to direct treatment selection are lacking apart from one randomized controlled trial assessing the use of thalidomide (3), and treatment decisions are based on case series and case reports (2,(4)(5)(6). First-line therapy usually consists of radiation (7), autologous stem cell transplantation (ASCT) (8-13), and lenalidomide-based therapies (14)(15)(16)(17). Literature exists on the use of lenalidomide and bortezomib in the relapsed setting; however, data on the treatment of relapsed patients using daratumumab, elotuzumab, pomalidomide, and car lzomib based therapies are scarce.…”

Section: Introductionmentioning

confidence: 99%

Daratumumab, Carfilzomib, Pomalidomide and Elotuzumab for the Treatment of POEMS Syndrome- The Mayo Clinic Experience

Vaxman

Kumar²,

Buadi

et al. 2022

Preprint

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POEMS syndrome is a rare paraneoplastic syndrome and therapies are directed against plasma cells that produce the proteins that cause this syndrome. Novel therapies are widely used in multiple myeloma aiming for plasma cell eradication. However, data on their use in POEMS syndrome are lacking. In this case series, we provide the Mayo Clinic experience in treating 16 patients with relapsed POEMS syndrome with novel agents (daratumumab, carfilzomib, pomalidomide, and elotuzumab). The patients were treated with a doublet including dexamethasone (N = 5) (31%) or in various combinations with other agents: DRd (N = 6), DC(V)d (N = 3), KRd (N = 3), KPd (N = 1), DP(V)d (N = 5), and EloRd (N = 1). The outcomes with novel agent therapies were favorable. Overall, twelve patients treated with daratumumab-based therapies (86%) and five patients treated with carfilzomib-based therapies (83%) responded to therapy. Among patients treated with daratumumab based therapies (N = 14), 9 patients achieved CR/VGPRH, 7 patients achieved CRV, and 5 patients achieved CRP. Among patients treated with carfilzomib-based therapies (N = 6), 3 patients achieved CR/VGPRH, and one achieved PRH. At a median follow-up of 38 months since starting of the novel agent (IQR 24–57), 15 of the patients (93%) are still alive, and the median TTNT was not reached. None of the patients discontinued therapy due to adverse events and no deaths occurred on therapy. Novel therapies were safe with 7 events of hospitalization due to pneumonia (4 in daratumumab-based therapies and 3 on carfilzomib-based therapies), and 4 patients were hospitalized due to volume overload. Three patients experienced infusion-related reactions (IRR) to the first dose of IV daratumumab. The response rate to novel agents was high, and the responses were deep. Novel agent therapies were safe, and no death case occurred on therapy. Future studies are needed to clarify the optimal sequence of novel agents and the best combination.

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

Cited by 3 publications

References 43 publications

Comprehensive Diagnosis and Management of POEMS Syndrome

Comprehensive Diagnosis and Management of POEMS Syndrome

Efficacy of novel agents in patients with nephropathy associated with POEMS syndrome

Daratumumab, Carfilzomib, Pomalidomide and Elotuzumab for the Treatment of POEMS Syndrome- The Mayo Clinic Experience

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