Long-term evaluation of physical improvement and survival of autologous stem cell transplantation in POEMS syndrome

Ohwada, Chikako; Sakaida, Emiko; Kawajiri-Manako, Chika; Nagao, Yuhei; Oshima-Hasegawa, Nagisa; Togasaki, Emi; Muto, Tomoya; Tsukamoto, Shokichi; Mitsukawa, Shio; Takeda, Yusuke; Mimura, Naoya; Takeuchi, Masahiro; Shimizu, Naomi; Misawa, Sonoko; Iseki, Tohru; Kuwabara, Satoshi; Nakaseko, Chiaki

doi:10.1182/blood-2017-07-795385

Cited by 39 publications

(54 citation statements)

References 15 publications

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“…To our knowledge, this is the first paper detailing the experience of ASCT for POEMS syndrome at a UK centre. This analysis confirms high rates of overall survival and PFS following transplantation (Karam et al, 2015;Kourelis et al, 2016a;Cook et al, 2017;Ohwada et al, 2018;D'Souza et al, 2012). Our experience confirms that attainment of HR CR following ASCT in POEMS syndrome is associated with a significantly lower rate of relapse (Kourelis et al, 2016b;Wang et al, 2017;Ohwada et al, 2018).…”

supporting

confidence: 86%

High‐dose therapy and autologous transplantation for POEMS Syndrome: effective, but how to optimise?

et al. 2019

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supporting

confidence: 86%

High‐dose therapy and autologous transplantation for POEMS Syndrome: effective, but how to optimise?

et al. 2019

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“…HDT with ASCT has been recommended as a first‐line therapy for younger patients with POEMS syndrome. Outstanding efficacy of HDT with ASCT in POEMS syndrome has been reported from a number of case series . The most remarkable effect of transplantation is rapid recovery for patients with severe neuropathy, even for patients who required a wheel chair .…”

Section: Treatmentmentioning

confidence: 99%

“…The most remarkable effect of transplantation is rapid recovery for patients with severe neuropathy, even for patients who required a wheel chair . Regarding long‐term outcomes, the 5‐year progression‐free survival and overall survival after HDT with ASCT has been reported as 63–76% and 90–97%, respectively . However, the disadvantages of HDT with ASCT must be considered.…”

Section: Treatmentmentioning

confidence: 99%

“…The first disadvantage is that patients with organ failure or massive effusions cannot receive immediate transplantation. These patients should receive pre‐transplantation induction therapy to safely undergo HDT with ASCT . Thalidomide, lenalidomide or bortezomib are candidates of induction therapy; however, long‐term treatment might cause myelosuppression, resulting in decreased efficiency of stem cell harvest, especially with lenalidomide .…”

Section: Treatmentmentioning

confidence: 99%

“…The third disadvantage is transplantation‐related complications and mortality. Engraftment syndrome is the most common complication, and its morbidity rate has been reported as 12–40% . We should carefully select candidates for HDT with ASCT, as the incidence of transplantation‐related mortality was 3–7% …”

Section: Treatmentmentioning

confidence: 99%

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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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Clonal immunoglobulin λ light‐chain gene rearrangements detected by next generation sequencing in POEMS syndrome

et al. 2018

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Polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes (POEMS) syndrome is a rare plasma cell dyscrasia characterized by polyneuropathy, organomegaly, endocrinopathy, extravascular fluid overload, M protein, and a myriad of skin changes. The pathogenesis is poorly understood, but monoclonal plasma cells are λ-restricted and these immunoglobulin λ light chain variable (IGLV) region genes are derived from only two germlines, either IGLV1-44 or 1-40. Here we analyzed the clonal IGLV gene rearrangements of genomic DNA samples of bone marrow mononuclear cells using next-generation sequencing (NGS) to understand the clonal composition of IGLV genes in patients with POEMS syndrome (n = 30). The dominant IGLV gene rearrangement of POEMS syndrome-specific germline sequences were significantly increased in 11 POEMS patients (36.7%; IGLV1-44: n = 9, IGLV1-40: n = 2). In some cases, IGLV gene rearrangement clone was not detected as significant increase but was detected using cDNA samples by heteroduplex (HD) analysis and Sanger sequencing, suggesting that the quite small number of monoclonal plasma cells may produce large quantity of mRNA of monoclonal proteins. However, significant increase of dominant clone sizes was not directly linked to the initial disease status. On the other hand, in cases with significantly increased dominant clones, they decreased and increased accompanying with disease remission and relapse. These data demonstrate that monoclonal plasma cells are related to the pathogenesis of POEMS syndrome.

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Long-term evaluation of physical improvement and survival of autologous stem cell transplantation in POEMS syndrome

Cited by 39 publications

References 15 publications

High‐dose therapy and autologous transplantation for POEMS Syndrome: effective, but how to optimise?

High‐dose therapy and autologous transplantation for POEMS Syndrome: effective, but how to optimise?

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

Clonal immunoglobulin λ light‐chain gene rearrangements detected by next generation sequencing in POEMS syndrome

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