POEMS Syndrome: Real World Experience in Diagnosis and Systemic Therapy - 108 Patients Multicenter Analysis

Jurczyszyn, Artur; Castillo, Jorge J.; Olszewska-Szopa, Magdalena; Kumar, Lalit; Thibaud, Santiago; Richter, Joshua; Flicker, Kari; Fiala, Mark A.; Vij, Ravi; Yi, Shuhua; Xu, Fang; Silbermann, Rebecca; Gaisán, C. Montes; Ocio, Enrique M.; Waszczuk‐Gajda, Anna; Crusoé, Edvan; Salomon-Perzyński, Aleksander; Hus, Iwona; Dávila‐Valls, Julio; Gozzetti, Alessandro; Czepiel, Jacek; Krzanowska, Katarzyna; Chappell, Aimee; Chellapuram, Santosh Kumar; Suska, Anna

doi:10.1016/j.clml.2021.10.007

Cited by 15 publications

(16 citation statements)

References 17 publications

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“…However, they are now less commonly used than other agents, and alkylators alone have the lowest CR H /VGPR H rates when compared with immunomodulators or proteasome inhibitor (PI) combinations (20% vs 44%-61%) in a study of 20 patients with up-front melphalan and 13 up-front cyclophosphamide. 26 Melphalan-dexamethasone has been studied in several series, with reported clinical response rates of 44%. 27 The prospective study of Mel/Dex enrolled 31 patients achieving a CR H of 39%.…”

Section: Ch E Moth Er a Py CL Asse S A N D Age N Ts Alkylatorsmentioning

confidence: 99%

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Evidence‐based medical treatment of POEMS syndrome

et al. 2022

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I N TRODUC TIONPOEMS syndrome is a rare multisystem paraneoplastic disorder characterised by Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal protein and Skin changes. The exact incidence is unknown. Patients may have diverse clinical presentations which are not found in the acronym, including extra-vascular overload, thrombosis and elevated raised serum vascular endothelial growth factor (VEGF). Full diagnostic criteria are outlined in Table 1. The diagnosis of POEMS syndrome is confirmed when both of the mandatory major criteria, one of the three other major criteria, and one of the six minor criteria are present. The underlying pathogenesis is not fully understood; however, an underlying slowly progressive low-level plasma cell dyscrasia is central alongside elevated VEGF. Most patients do not meet criteria for myeloma, with less than 5% plasma cell infiltration being the median infiltrate in a series of 67 patients, 1 on a background of polytypic plasma cells, as well as megakaryocyte hyperplasia, clustering and atypia. Delayed diagnosis is very common, of up to two years, 2 which may lead to organ dysfunction and subsequent adverse outcomes. 3 However, treatment options are highly effective at eradicating the underlying clone. Due to the rarity of POEMS, there are limited trial data to guide treatment decisions and no international consensus guidelines. Data are largely gleaned from retrospective case series. Therapeutic options are adapted from those used in multiple myeloma and solitary plasmacytoma. Other reviews have looked at the overall management of POEMS 4 ; here we focus specifically on medical treatment options, and present the first systematic review of current available data. Treatment aimsTreatment is primarily directed at eradicating the underlying plasma cell dyscrasia. For most patients, treatment is highly effective in halting disease progression. The aims of treatment are prolongation of overall survival (OS), increased duration of remission (progression-free survival, PFS) and

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Section: Ch E Moth Er a Py CL Asse S A N D Age N Ts Alkylatorsmentioning

confidence: 99%

“…Its benefit has been described in single cases. 26,39 Ixazomib is an oral PI which is theoretically attractive due to low neurotoxicity rates and oral administration. It is however the least active of the three available PIs.…”

Section: Proteasome Inhibitorsmentioning

confidence: 99%

Evidence‐based medical treatment of POEMS syndrome

et al. 2022

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“…In the myeloma context, bortezomib has been shown to have equal efficacy on a weekly rather than biweekly schedule and subcutaneous rather than intravenous with additional reduced rates of peripheral neuropathy [6,7]. with no treatment discontinuation [9].…”

Section: Discussionmentioning

confidence: 99%

“…The largest series of bortezomib–dexamethasone use in POEMS recently reported on 69 patients at 1.3 mg/m 2 subcutaneously [ 8 ] with excellent outcomes (haematological complete remission rate 46%, VEGF complete remission rate 71%, neurological improvement 88%, 2‐year time to next treatment 66%) and only 3% developed grade 1 bortezomib‐induced neuropathy after six cycles of treatment, which was reversible after drug withdrawal. A multicentre study comparing upfront therapies showed that bortezomib was the most efficacious treatment (69% haematological complete remission/very good partial response compared with immunomodulatory drugs and alkylators) with no treatment discontinuation [ 9 ].…”

Section: Discussionmentioning

confidence: 99%

Daratumumab–bortezomib–dexamethasone use in relapsed POEMS syndrome

Khwaja

Keh

Smyth

et al. 2022

eJHaem

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POEMS syndrome is a rareparaneoplastic disorder driven by an underlying low level plasma cell dyscrasiaand associated with elevated serum vascular endothelial growth factor (VEGF). Dueto its rarity, there are no internationally agreed standards of care, with verylimited data to guide management in the relapse setting. Agents used in myelomaare rational choices and have been employed. Daratumumab has been reported intwo case studies with lenalidomide-dexamethasone, one in the upfront and one inthe relapsed setting. We are the first to report here three cases ofdaratumumabbortezomib-dexamethasone (DVd) use in relapsed POEMS postautologous stem cell transplant with good VEGF and clinical responses. Our casesadd to the literature on efficacy of daratumumab and are the first to report onits safe use with bortezomib in relapsed POEMS. It should be considered as aclinical option, in patients not responding to conventional first linetherapies.

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“…Thus, utilization of bortezomib as frontline monotherapy appears to be tolerated and highly effective. [2] In relapse cases, other treatment options were tested in which the combination of bortezomib and dexamethasone was given together with daratumumab, an immunomodulatory drug [3] or cyclophosphamide. On the other hand, other publications reported the use of bortezomib and dexamethasone only as a first-line regimen for newly diagnosed POEMS syndrome and a majority of these patients include Chinese males in their fifth decade of life.…”

Section: Introductionmentioning

confidence: 99%

Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy and Skin abnormalities (POEMS) Syndrome in a Filipino Nurse Returning to Work Following Bortezomib Treatment: A Case Report

Endaya¹,

Rosales²

2022

JMUST

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POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes) also known as Crow-Fukase syndrome or Takatsuki syndrome is a rare and disabling paraneoplastic syndrome that frequently occurs in the fifth or sixth decade of life without a known standard first-line therapy. A 34-year-old Filipino male nurse, who presented with gradually progressive distal lower extremity weakness and sharp shooting pain in bilateral legs accompanied by mild joint effusion on the left knee, hypertrichosis, bilateral conjunctival injection, and gynecomastia underwent extensive workup and was diagnosed with POEMS syndrome. Complete blood count revealed erythrocytosis and thrombocytosis with elevated serum VEGF (vascular endothelial growth factor) and elevated monoclonal serum free lambda light chains. The electrophysiologic studies revealed chronic demyelinating sensorimotor polyneuropathy while bone marrow core biopsy and bone marrow aspirate smear immunohistochemical staining showed it to be positive for lambda and CD138. He had an initial unsuccessful treatment course with melphalan and prednisone. Hence, bortezomib and dexamethasone were given which gave significant improvement in symptoms from the overall neuropathy limitation score of 5 to 1.

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POEMS Syndrome: Real World Experience in Diagnosis and Systemic Therapy - 108 Patients Multicenter Analysis

Cited by 15 publications

References 17 publications

Evidence‐based medical treatment of POEMS syndrome

Evidence‐based medical treatment of POEMS syndrome

Daratumumab–bortezomib–dexamethasone use in relapsed POEMS syndrome

Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy and Skin abnormalities (POEMS) Syndrome in a Filipino Nurse Returning to Work Following Bortezomib Treatment: A Case Report

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