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Angela Dispenzieri - One of the best experts on this subject based on the ideXlab platform.
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ixazomib lenalidomide and dexamethasone for patients with POEMS Syndrome
Journal of Clinical Oncology, 2019Co-Authors: Angela Dispenzieri, Michelle L Mauermann, Martha Q Lacy, Betsy Laplant, Prashant Kapoor, Nelson Leung, Yi Lisa Hwa, A L Fonder, Francis K Buadi, Suzanne R HaymanAbstract:8019Background: POEMS Syndrome is a rare paraneoplastic Syndrome caused by an underlying plasma cell disorder. Most of the information regarding treatment has been gleaned from retrospective data. ...
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POEMS Syndrome 2017 update on diagnosis risk stratification and management
American Journal of Hematology, 2017Co-Authors: Angela DispenzieriAbstract:Disease Overview 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 3 of the major criteria, two of which must include polyradiculoneuropathy and clonal plasma cell disorder, 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. The number of clinical criteria is not prognostic, but the extent of the plasma cell disorder is. Those patients with an iliac crest bone marrow biopsy that does not reveal a plasma cell clone are candidates for local radiation therapy; those with a more extensive or disseminated clone will be candidates for systemic therapy 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 3–6 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. The benefit of anti-VEGF antibodies is conflicting. 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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thrombocytosis distinguishes POEMS Syndrome from chronic inflammatory demyelinating polyneuropathy
Muscle & Nerve, 2015Co-Authors: Elie Naddaf, Angela Dispenzieri, Jayawant N Mandrekar, Michelle L MauermannAbstract:Introduction POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, and skin changes) Syndrome may be mistaken for chronic inflammatory demyelinating polyneuropathy (CIDP). Differentiating the 2 entities is crucial, as there are major treatment implications. Methods We compared platelet counts in 136 POEMS patients and 67 CIDP controls. Results Of the patients with POEMS, 53.7% had thrombocytosis, compared with 1.5% of those with CIDP (P < 0.0001). The median platelet count in patients with POEMS was 467,000/μl compared with 275,000/μl in those with CIDP (P < 0.0001). Conclusions Thrombocytosis is a helpful indicator to prompt clinicians to consider the diagnosis of POEMS Syndrome in patients who are thought to have CIDP, and is an important reminder of the increased risk of thrombotic events in POEMS Syndrome. Muscle Nerve 52: 658–659, 2015
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POEMS Syndrome 2014 update on diagnosis risk stratification and management
American Journal of Hematology, 2014Co-Authors: Angela DispenzieriAbstract:Disease overview: 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. The number of clinical criteria is not prognostic, but the extent of the PCD is. Those patients with an iliac crest bone marrow biopsy that does not reveal a plasma cell clone are candidates for local radiation therapy; those with a more extensive or disseminated clone will be candidates for systemic therapy. 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 3–6 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. The benefit of anti-VEGF antibodies is conflicting. 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. Am. J. Hematol. 89:214–223, 2014. © 2014 Wiley Periodicals, Inc.
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POEMS Syndrome update on diagnosis risk stratification and management
American Journal of Hematology, 2012Co-Authors: Angela DispenzieriAbstract:Disease overview: 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 plasma cell disorder, 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. The number of clinical criteria is not prognostic, but the extent of the plasma cell disorder is. Those patients with an iliac crest bone marrow biopsy that does not reveal a plasma cell clone are candidates for local radiation therapy; those with a more extensive or disseminated clone will be candidates for systemic therapy. 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 3–6 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. The benefit of anti-VEGF antibodies is conflicting. Prompt recognition and institution of both supportive care measures and therapy directed against the plasma cell result in the best outcomes. Am. J. Hematol. 90:951–962, 2015. © 2015 Wiley Periodicals, Inc.
Satoshi Kuwabara - One of the best experts on this subject based on the ideXlab platform.
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abstract 20049 transthoracic echocardiographical tte cardiac manifestations of the polyneuropathy organomegaly endocrinopathy monoclonal gammopathy and skin changes POEMS Syndrome
Circulation, 2017Co-Authors: Nobusada Funabashi, Satoshi Kuwabara, Sonoko Misawa, Hiroyuki Takaoka, Koya Ozawa, Yoshio KobayashiAbstract:Introduction: POEMS Syndrome is a rare multisystem disease associated with plasma cell dyscrasia, and with increased levels of vascular endothelial growth factor (VEGF). Hypothesis: Some patients h...
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NEOPLASIA Brief report Restrictive usage of monoclonal immunoglobulin light chain germline in POEMS Syndrome
2016Co-Authors: Satoshi Kuwabara, Yasushi SaitoAbstract:POEMS Syndrome is a rare plasma cell disorder characterized by peripheral neu-ropathy, monoclonal gammopathy, and high levels of serum vascular endothelial growth factor, the pathogenesis of which remains unclear. A unique feature of this Syndrome is that the proliferating mono-clonal plasma cells are essentially -restricted. Here we determined com-plete nucleotide sequences of monoclo-nal immunoglobulin light chain (IGL) variable regions in 11 patients with POEMS Syndrome. The V-region of the Ig gene of all 11 patients was restricted to the V1 subfamily. Searching for ho-mologies with IGL germlines revealed that 2 germlines, IGLV1-44*01 (9/11) and IGLV1-40*01 (2/10), were identified, with an average homology of 91.1%. The IGLJ3*02 gene was used in 11 of 11 re-arrangements with an average homology of 92.2%. These data suggest that the highly restricted use of IGL V1 germ-lines plays an important role in the patho-genesis of POEMS Syndrome. (Blood. 2008;112:836-839
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Altered axonal excitability properties and nerve edema in POEMS Syndrome
Clinical neurophysiology : official journal of the International Federation of Clinical Neurophysiology, 2015Co-Authors: Satsuki Mitsuma, Sonoko Misawa, Sagiri Isose, Kazumoto Shibuya, Yukari Sekiguchi, Yuta Iwai, Minako Beppu, Keisuke Watanabe, Hiroshi Amino, Satoshi KuwabaraAbstract:Abstract Objective POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes) Syndrome is a rare cause of demyelinating neuropathy with upregulation of vascular endothelial growth factor (VEGF). This study aimed to elucidate axonal excitability properties and their relation to VEGF levels and nerve edema in POEMS neuropathy. Methods Axonal excitability measurement and nerve ultrasound were performed in the median nerve of 33 patients with POEMS Syndrome. Serum VEGF levels were measured by ELISA. Results Compared with normal subjects ( n =87), POEMS patients showed longer strength-duration time constant, fanning-out of threshold electrotonus curves, and greater threshold changes in a hyperpolarizing current–threshold relationship. Nerve ultrasound showed significant enlargement in POEMS patients. Serum VEGF levels and the extent of nerve edema partly correlated with nerve conduction slowing, as well as persistent sodium currents and inward rectification. Conclusions In POEMS Syndrome, patterns of changes in excitability properties could suggest increased persistent sodium currents, and impaired potassium and inward rectifying channels. The findings were not consistent with depolarization due to nerve edema and compression ischemia. Significance In addition to demyelination, nerve edema induced by upregulated VEGF, and upregulated inflammatory cytokines could modulate profiles of POEMS neuropathy.
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different neurological and physiological profiles in POEMS Syndrome and chronic inflammatory demyelinating polyneuropathy
Journal of Neurology Neurosurgery and Psychiatry, 2012Co-Authors: Saiko Nasu, Sonoko Misawa, Kazumoto Shibuya, Yukari Sekiguchi, Satsuki Mitsuma, Kazuaki Kanai, Yumi Fujimaki, Shigeki Ohmori, Shunsuke Koga, Satoshi KuwabaraAbstract:Background POEMS (polyneuropathy, organomegaly, endocrinopathy, M protein and skin changes) Syndrome, a rare cause of demyelinating neuropathy associated with multiorgan involvement, has been increasingly recognised. Polyneuropathy is often an initial manifestation and therefore the disorder can be misdiagnosed as chronic inflammatory demyelinating polyneuropathy (CIDP). Objective To elucidate whether POEMS Syndrome and CIDP are differentiated based on profiles of neuropathy. Methods Clinical and electrophysiological data were reviewed in consecutive POEMS Syndrome (n=51) and typical CIDP (n=46) patients in a single Japanese hospital between 2000 and 2010. Results Both POEMS and CIDP patients showed symmetric polyneuropathy, physiological evidence of demyelination (70% of POEMS patients fulfilled the electrodiagnostic criteria for definite CIDP) and albuminocytological dissociation; 49% of the POEMS Syndrome patients had neuropathy onset and 60% of them were initially diagnosed as having CIDP by neurologists. Clinically, POEMS neuropathy more frequently showed severe leg pain (76% vs 7%; p Conclusions Before development of typical systemic manifestations, POEMS neuropathy can be distinguished from CIDP by the clinical profile and patterns of nerve conduction abnormalities. Recognition of these features leads to early diagnosis and proper treatment for POEMS Syndrome.
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neurologic improvement after peripheral blood stem cell transplantation in POEMS Syndrome
Neurology, 2008Co-Authors: Satoshi Kuwabara, Sonoko Misawa, Takamichi Hattori, Kazuaki Kanai, Setsu Sawai, Motoi Nishimura, Yo Ichi Suzuki, Yuriko Kikkawa, Chiaki NakasekoAbstract:Background: Polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes (POEMS) Syndrome is a rare multisystem disorder associated with plasma cell dyscrasia. There is increasing evidence that high-dose chemotherapy with autologous peripheral blood stem cell transplantation (Auto-PBSCT) is an efficacious treatment. Objective: To elucidate the extent and time course of neurologic improvement after Auto-PBSCT in patients with POEMS Syndrome. Methods: Clinical and electrophysiologic findings in nine patients were reviewed. The median follow-up period was 20 months (range, 8 to 49 months). Serum levels of vascular endothelial growth factor (VEGF) were measured by ELISA. Results: Serum VEGF levels rapidly decreased a month after Auto-PBSCT. Within 3 months, neurologic improvement began, and all the patients showed substantial neurologic recovery during the next 3 months. Particularly, three initially chairbound patients regained ability to walk at 6 months. Nerve conduction studies showed significant increases in conduction velocities and amplitudes within 6 months of treatment. At the end of follow-up periods, neuropathy was still improving, and no patients had recurrence of symptoms. Conclusion: Autologous peripheral blood stem cell transplantation results in obvious neurologic improvement within 6 months, presumably by extensive axonal regeneration and remyelination. This therapy could be considered as a first line treatment for patients with polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes Syndrome with younger onset even if they are tetraplegic.
Sonoko Misawa - One of the best experts on this subject based on the ideXlab platform.
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Cerebral large artery stenosis and occlusion in POEMS Syndrome
'Springer Science and Business Media LLC', 2021Co-Authors: Atsuhiko Sugiyama, Sonoko Misawa, Yukari Sekiguchi, Hajime Yokota, Hiroki Mukai, Kyosuke Koide, Tomoki Suichi, Jun Matsushima, Takashi Kishimoto, Zen-ichi TaneiAbstract:Abstract Background This study aimed to investigate the frequency and risk factors for cerebral artery stenosis and occlusion in patients with polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes (POEMS) Syndrome. Methods We reviewed results of magnetic resonance angiography (MRA) or computed tomography angiography (CTA) in 61 patients with POEMS Syndrome seen between 2010 and 2017. Stenosis or occlusion was assessed in the initial MRA/CTA. Multivariate analysis was used to identify risk factors for artery stenosis/occlusion. In an autopsy case, pathologic examination was conducted of the occluded middle cerebral arteries. Results Stenosis (> 50 %) or occlusion of the major cerebral arteries was found in 29 (47.5 %) patients on the initial MRA/CTA. The internal carotid artery was involved most frequently (32.8 %), followed by the anterior (21.3 %) and middle (16.4 %) cerebral arteries. The basilar (1.3 %) and vertebral (3.6 %) arteries were rarely affected. Cerebral infarction developed in eight (13.1 %) patients. The serum vascular endothelial growth factor (VEGF) level was an independent predictor for stenosis/occlusion (odds ratio, 1.228; 95 % confidence interval, 1.042–1.447; P = 0.014). An autopsy study showed occluded middle cerebral arteries by fibrous and myxomatous thickening of intima with splitting of the internal elastic lamina. Follow-up MRA in 23 patients showed improved, worsened, and unchanged stenosis in 20.7 %, 8.7 %, and 69.6 %, respectively. Conclusions Cerebral large-vessel stenosis or occlusion is frequently seen in approximately half of patients with POEMS Syndrome. Vasculopathy was related to serum VEGF levels and thereby disease activity. Assessment of cerebral vessels is recommended in these patients to improve management
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abstract 20049 transthoracic echocardiographical tte cardiac manifestations of the polyneuropathy organomegaly endocrinopathy monoclonal gammopathy and skin changes POEMS Syndrome
Circulation, 2017Co-Authors: Nobusada Funabashi, Satoshi Kuwabara, Sonoko Misawa, Hiroyuki Takaoka, Koya Ozawa, Yoshio KobayashiAbstract:Introduction: POEMS Syndrome is a rare multisystem disease associated with plasma cell dyscrasia, and with increased levels of vascular endothelial growth factor (VEGF). Hypothesis: Some patients h...
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safety and efficacy of thalidomide in patients with POEMS Syndrome a multicentre randomised double blind placebo controlled trial
Lancet Neurology, 2016Co-Authors: Sonoko Misawa, Yukari Sekiguchi, Yasunori Sato, Kanako Katayama, Kengo Nagashima, Reiko Aoyagi, Gen Sobue, Haruki Koike, Ichiro Yabe, Hidenao SasakiAbstract:Summary Background Polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes (POEMS) Syndrome is a rare cause of demyelinating neuropathy, with multi-organ involvement characterised by plasma cell dyscrasia and VEGF overproduction. No treatments have been established for patients with POEMS Syndrome who are not eligible for stem-cell transplantation. Thalidomide suppresses VEGF and plasma cell proliferation. We aimed to assess the safety and efficacy of thalidomide for the treatment of POEMS Syndrome. Methods We did a randomised, double-blind, placebo-controlled, phase 2/3 trial at 12 hospitals in Japan. Adults (age ≥20 years) with POEMS Syndrome who were ineligible for autotransplantation were randomly assigned (1:1) by a minimisation method to treatment with oral dexamethasone (12 mg/m 2 per day on the first 4 days of every 28-day cycle) plus either oral thalidomide (200 mg daily) or placebo for six cycles. All study personnel and patients were masked to treatment allocation. The primary endpoint was the reduction rate of serum VEGF concentrations at 24 weeks. Analysis was by intention to treat. This study is registered with the UMIN Clinical Trials Registry, UMIN000004179. Findings Between Nov 11, 2010, and July 3, 2014, we randomly assigned 25 patients to receive either thalidomide (n=13) or placebo (n=12); one patient in the placebo group was excluded from analyses because of a protocol violation. The adjusted mean VEGF concentration reduction rate at 24 weeks was 0·39 (SD 0·34) in the thalidomide group compared with −0·02 (0·54) in the placebo group (adjusted mean difference 0·41, 95% CI 0·02–0·80; p=0·04). Mild sinus bradycardia was more frequent in the thalidomide group than in the placebo group (seven [54%] vs zero; p=0·006). Five patients had serious adverse events: three in the thalidomide group (transient cardiac arrest, heart failure, and dehydration) and two in the placebo group (ileus and fever). No deaths occurred during the randomised study. In the 48-week open-label study period (n=22), newly developed adverse events were sinus bradycardia (n=4), constipation (n=5), and mild sensory neuropathy (n=5). Two patients died in the open-label study; both patients were initially in the placebo group, and the cause of death was progression of the disease. Interpretation Thalidomide reduces serum VEGF concentrations and represents a new treatment for patients with POEMS Syndrome who are not eligible for stem-cell transplantation. Thalidomide treatment poses a risk of bradycardia; however, the benefits are likely to exceed the risk. Funding Japanese Ministry of Health, Labour, and Welfare, and Fujimoto Pharmaceuticals.
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vascular endothelial growth factor as a predictive marker for POEMS Syndrome treatment response retrospective cohort study
BMJ Open, 2015Co-Authors: Sonoko Misawa, Kazumoto Shibuya, Yukari Sekiguchi, Minako Beppu, Setsu Sawai, Yasunori Sato, Katsuhiro Katayama, Hideki Hanaoka, Fumio Nomura, Yuta IwaiAbstract:Objective POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes) Syndrome is a rare multisystem disease characterised by plasma cell dyscrasia and overproduction of vascular endothelial growth factor (VEGF). VEGF is assumed to be useful in monitoring disease activity, because VEGF levels usually decrease after treatment. However, there is no study to investigate whether the extent of decrease in VEGF correlates with clinical outcome. We tested the predictive efficacy of serum VEGF levels in POEMS Syndrome. Method This was an institutional review board approved retrospective observational cohort study of 20 patients with POEMS monitored regularly for more than 12 months (median follow-up, 87 months) after treatment onset using our prospectively accumulated database of POEMS from 1999 to 2015. Patients were treated by autologous peripheral blood stem cell transplantation or thalidomide administration. Serum VEGF was measured by ELISA. Outcome measures included clinical and laboratory findings and relapse-free survival. Results Serum VEGF levels decreased rapidly after treatment, and stabilised by 6 months post treatment. Patients with normalised serum VEGF levels ( Conclusions Serum VEGF level at 6 months post treatment is a predicative biomarker for disease activity and prognosis in POEMS Syndrome. Serum VEGF could be used as a surrogate endpoint for relapse-free survival or clinical or laboratory improvement of POEMS Syndrome for clinical trials.
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Altered axonal excitability properties and nerve edema in POEMS Syndrome
Clinical neurophysiology : official journal of the International Federation of Clinical Neurophysiology, 2015Co-Authors: Satsuki Mitsuma, Sonoko Misawa, Sagiri Isose, Kazumoto Shibuya, Yukari Sekiguchi, Yuta Iwai, Minako Beppu, Keisuke Watanabe, Hiroshi Amino, Satoshi KuwabaraAbstract:Abstract Objective POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes) Syndrome is a rare cause of demyelinating neuropathy with upregulation of vascular endothelial growth factor (VEGF). This study aimed to elucidate axonal excitability properties and their relation to VEGF levels and nerve edema in POEMS neuropathy. Methods Axonal excitability measurement and nerve ultrasound were performed in the median nerve of 33 patients with POEMS Syndrome. Serum VEGF levels were measured by ELISA. Results Compared with normal subjects ( n =87), POEMS patients showed longer strength-duration time constant, fanning-out of threshold electrotonus curves, and greater threshold changes in a hyperpolarizing current–threshold relationship. Nerve ultrasound showed significant enlargement in POEMS patients. Serum VEGF levels and the extent of nerve edema partly correlated with nerve conduction slowing, as well as persistent sodium currents and inward rectification. Conclusions In POEMS Syndrome, patterns of changes in excitability properties could suggest increased persistent sodium currents, and impaired potassium and inward rectifying channels. The findings were not consistent with depolarization due to nerve edema and compression ischemia. Significance In addition to demyelination, nerve edema induced by upregulated VEGF, and upregulated inflammatory cytokines could modulate profiles of POEMS neuropathy.
Kazuaki Kanai - One of the best experts on this subject based on the ideXlab platform.
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markedly upregulated serum interleukin 12 as a novel biomarker in POEMS Syndrome
Neurology, 2012Co-Authors: Kazuaki Kanai, Sonoko Misawa, Sagiri Isose, Kazumoto Shibuya, Setsu Sawai, Kazuyuki Sogawa, Masahiro Mori, Yumi Fujimaki, Yuichi Noto, Yukari SekiguchiAbstract:Objective: To systematically study abnormalities in cytokine profiles in polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes (POEMS) Syndrome, which has been increasingly recognized as a cause of demyelinating neuropathy associated with plasma cell dyscrasia and elevated serum level of vascular endothelial growth factor (VEGF). Methods: In this case-control study, we measured serum levels of 27 cytokines in patients with POEMS Syndrome using a multiplex suspension array system, and compared them with those of controls. In 10 patients, serial changes after treatment were analyzed. Results: Interleukin (IL)–12 as well as VEGF levels were markedly increased ( p p > 0.01 and p > 0.05, respectively). Conclusions: Our findings suggest that serum IL-12 is a biomarker of the disease activity in POEMS Syndrome. The overproduction of IL-12, as well as VEGF, is likely to play an important role in the pathogenesis of the disorder, and could contribute to the peripheral nerve demyelination in POEMS Syndrome.
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different neurological and physiological profiles in POEMS Syndrome and chronic inflammatory demyelinating polyneuropathy
Journal of Neurology Neurosurgery and Psychiatry, 2012Co-Authors: Saiko Nasu, Sonoko Misawa, Kazumoto Shibuya, Yukari Sekiguchi, Satsuki Mitsuma, Kazuaki Kanai, Yumi Fujimaki, Shigeki Ohmori, Shunsuke Koga, Satoshi KuwabaraAbstract:Background POEMS (polyneuropathy, organomegaly, endocrinopathy, M protein and skin changes) Syndrome, a rare cause of demyelinating neuropathy associated with multiorgan involvement, has been increasingly recognised. Polyneuropathy is often an initial manifestation and therefore the disorder can be misdiagnosed as chronic inflammatory demyelinating polyneuropathy (CIDP). Objective To elucidate whether POEMS Syndrome and CIDP are differentiated based on profiles of neuropathy. Methods Clinical and electrophysiological data were reviewed in consecutive POEMS Syndrome (n=51) and typical CIDP (n=46) patients in a single Japanese hospital between 2000 and 2010. Results Both POEMS and CIDP patients showed symmetric polyneuropathy, physiological evidence of demyelination (70% of POEMS patients fulfilled the electrodiagnostic criteria for definite CIDP) and albuminocytological dissociation; 49% of the POEMS Syndrome patients had neuropathy onset and 60% of them were initially diagnosed as having CIDP by neurologists. Clinically, POEMS neuropathy more frequently showed severe leg pain (76% vs 7%; p Conclusions Before development of typical systemic manifestations, POEMS neuropathy can be distinguished from CIDP by the clinical profile and patterns of nerve conduction abnormalities. Recognition of these features leads to early diagnosis and proper treatment for POEMS Syndrome.
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neurologic improvement after peripheral blood stem cell transplantation in POEMS Syndrome
Neurology, 2008Co-Authors: Satoshi Kuwabara, Sonoko Misawa, Takamichi Hattori, Kazuaki Kanai, Setsu Sawai, Motoi Nishimura, Yo Ichi Suzuki, Yuriko Kikkawa, Chiaki NakasekoAbstract:Background: Polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes (POEMS) Syndrome is a rare multisystem disorder associated with plasma cell dyscrasia. There is increasing evidence that high-dose chemotherapy with autologous peripheral blood stem cell transplantation (Auto-PBSCT) is an efficacious treatment. Objective: To elucidate the extent and time course of neurologic improvement after Auto-PBSCT in patients with POEMS Syndrome. Methods: Clinical and electrophysiologic findings in nine patients were reviewed. The median follow-up period was 20 months (range, 8 to 49 months). Serum levels of vascular endothelial growth factor (VEGF) were measured by ELISA. Results: Serum VEGF levels rapidly decreased a month after Auto-PBSCT. Within 3 months, neurologic improvement began, and all the patients showed substantial neurologic recovery during the next 3 months. Particularly, three initially chairbound patients regained ability to walk at 6 months. Nerve conduction studies showed significant increases in conduction velocities and amplitudes within 6 months of treatment. At the end of follow-up periods, neuropathy was still improving, and no patients had recurrence of symptoms. Conclusion: Autologous peripheral blood stem cell transplantation results in obvious neurologic improvement within 6 months, presumably by extensive axonal regeneration and remyelination. This therapy could be considered as a first line treatment for patients with polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes Syndrome with younger onset even if they are tetraplegic.
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thalidomide reduces serum vegf levels and improves peripheral neuropathy in POEMS Syndrome
Journal of Neurology Neurosurgery and Psychiatry, 2008Co-Authors: Satoshi Kuwabara, Sonoko Misawa, Takamichi Hattori, Kazuaki Kanai, Setsu Sawai, Motoi Nishimura, Chiaki NakasekoAbstract:Background: Polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes (POEMS) Syndrome is a rare multi-system disorder associated with plasma-cell dyscrasia. Several case series and reports have suggested that high-dose chemotherapy with autologous peripheral blood stem-cell transplantation is efficacious treatment, but this transplantation is not indicated for elderly patients and patients with renal failure. Objective: To investigate the effects of thalidomide treatment for POEMS Syndrome. Methods: Nine patients, who were not indicated for high-dose chemotherapy, were treated with thalidomide. Neurological disability scores, nerve conduction studies and serum levels of vascular endothelial growth factor (VEGF) were prospectively examined. VEGF levels were measured by an enzyme-linked immunosorbent assay. Results: During follow-up periods of 8–23 months (mean, 15 months), all patients showed substantial clinical improvement (n = 6) or stabilisation of symptoms (n = 3). Serum VEGF levels decreased in all patients and were normalised in five patients. Nerve conduction velocities in the median nerve increased in seven patients. There were no serious adverse effects, including thalidomide neuropathy. Conclusion: Thalidomide treatment should be further studied as a treatment for POEMS Syndrome, particularly for patients who are not indicated for transplantation therapy.
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patterns of nerve conduction abnormalities in POEMS Syndrome
Muscle & Nerve, 2002Co-Authors: Jia Ying Sung, Satoshi Kuwabara, Kazue Ogawara, Kazuaki Kanai, Takamichi HattoriAbstract:Polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes (POEMS) Syndrome is a rare cause of demyelinating and axonal neuropathy. POEMS Syndrome and chronic inflammatory demyelinating polyneuropathy (CIDP) cause peripheral nerve demyelination, and the electrodiagnostic findings may therefore be similar, but the two disorders are distinct. To elucidate the electrodiagnostic features of POEMS Syndrome, we reviewed nerve conduction studies of 8 patients, and compared their results with those in 42 patients with CIDP. The patients with POEMS Syndrome showed (1) slowing of nerve conduction that was more predominant in the intermediate than distal nerve segments, (2) rare conduction block (6% of the tested nerves), and (3) more severe attenuation of compound muscle action potentials in the lower than upper limbs. Findings in the CIDP patients were characterized by multifocal conduction slowing that was occasionally dominant distally, frequent conduction block (44% of tested nerves), and less discrepancy between upper and lower limb nerves. The pattern of nerve conduction abnormalities differs between these disorders. Recognition of these typical patterns may be helpful for early diagnosis of POEMS Syndrome.
Chiaki Nakaseko - One of the best experts on this subject based on the ideXlab platform.
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neurologic improvement after peripheral blood stem cell transplantation in POEMS Syndrome
Neurology, 2008Co-Authors: Satoshi Kuwabara, Sonoko Misawa, Takamichi Hattori, Kazuaki Kanai, Setsu Sawai, Motoi Nishimura, Yo Ichi Suzuki, Yuriko Kikkawa, Chiaki NakasekoAbstract:Background: Polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes (POEMS) Syndrome is a rare multisystem disorder associated with plasma cell dyscrasia. There is increasing evidence that high-dose chemotherapy with autologous peripheral blood stem cell transplantation (Auto-PBSCT) is an efficacious treatment. Objective: To elucidate the extent and time course of neurologic improvement after Auto-PBSCT in patients with POEMS Syndrome. Methods: Clinical and electrophysiologic findings in nine patients were reviewed. The median follow-up period was 20 months (range, 8 to 49 months). Serum levels of vascular endothelial growth factor (VEGF) were measured by ELISA. Results: Serum VEGF levels rapidly decreased a month after Auto-PBSCT. Within 3 months, neurologic improvement began, and all the patients showed substantial neurologic recovery during the next 3 months. Particularly, three initially chairbound patients regained ability to walk at 6 months. Nerve conduction studies showed significant increases in conduction velocities and amplitudes within 6 months of treatment. At the end of follow-up periods, neuropathy was still improving, and no patients had recurrence of symptoms. Conclusion: Autologous peripheral blood stem cell transplantation results in obvious neurologic improvement within 6 months, presumably by extensive axonal regeneration and remyelination. This therapy could be considered as a first line treatment for patients with polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes Syndrome with younger onset even if they are tetraplegic.
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thalidomide reduces serum vegf levels and improves peripheral neuropathy in POEMS Syndrome
Journal of Neurology Neurosurgery and Psychiatry, 2008Co-Authors: Satoshi Kuwabara, Sonoko Misawa, Takamichi Hattori, Kazuaki Kanai, Setsu Sawai, Motoi Nishimura, Chiaki NakasekoAbstract:Background: Polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes (POEMS) Syndrome is a rare multi-system disorder associated with plasma-cell dyscrasia. Several case series and reports have suggested that high-dose chemotherapy with autologous peripheral blood stem-cell transplantation is efficacious treatment, but this transplantation is not indicated for elderly patients and patients with renal failure. Objective: To investigate the effects of thalidomide treatment for POEMS Syndrome. Methods: Nine patients, who were not indicated for high-dose chemotherapy, were treated with thalidomide. Neurological disability scores, nerve conduction studies and serum levels of vascular endothelial growth factor (VEGF) were prospectively examined. VEGF levels were measured by an enzyme-linked immunosorbent assay. Results: During follow-up periods of 8–23 months (mean, 15 months), all patients showed substantial clinical improvement (n = 6) or stabilisation of symptoms (n = 3). Serum VEGF levels decreased in all patients and were normalised in five patients. Nerve conduction velocities in the median nerve increased in seven patients. There were no serious adverse effects, including thalidomide neuropathy. Conclusion: Thalidomide treatment should be further studied as a treatment for POEMS Syndrome, particularly for patients who are not indicated for transplantation therapy.