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POEMS Syndrome (Syndrome, POEMS)

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A multisystemic disorder characterized by a sensorimotor polyneuropathy (POLYNEUROPATHIES), organomegaly, endocrinopathy, monoclonal gammopathy, and pigmentary skin changes. Other clinical features which may be present include EDEMA; CACHEXIA; microangiopathic glomerulopathy; pulmonary hypertension (HYPERTENSION, PULMONARY); cutaneous necrosis; THROMBOCYTOSIS; and POLYCYTHEMIA. This disorder is frequently associated with osteosclerotic myeloma. (From Adams et al., Principles of Neurology, 6th ed, p1335; Rev Med Interne 1997;18(7):553-62)

Also Known As:

Syndrome, POEMS; Syndrome, Crow-Fukase; Polyneuropathy Organomegaly; Polyneuropathy, Organomegaly, Endocrinopathy, M Protein, and Skin Changes Syndrome; Crow Fukase Syndrome; Organomegalies, Polyneuropathy; Organomegaly, Polyneuropathy; Polyneuropathy Organomegalies; Syndrome, Takatsuki's; Takatsuki Syndrome; Crow-Fukase Syndrome; Takatsuki's Syndrome

Networked: 381 relevant articles (35 outcomes, 21 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	POEMS Syndrome (Syndrome, POEMS)
2.	Pulmonary Hypertension (Ayerza Syndrome)
3.	Polyneuropathies (Polyneuropathy)
4.	Plasmacytoma
5.	Edema

Experts

1.	Kuwabara, Satoshi: 17 articles (10/2015 - 01/2007)
2.	Dispenzieri, Angela: 17 articles (05/2015 - 11/2004)
3.	Misawa, Sonoko: 14 articles (10/2015 - 01/2007)
4.	Li, Jian: 10 articles (10/2015 - 06/2011)
5.	Kyle, Robert A: 8 articles (03/2015 - 11/2004)
6.	Misawa, S: 8 articles (01/2015 - 01/2006)
7.	Nakaseko, C: 8 articles (01/2015 - 01/2006)
8.	Kuwabara, S: 8 articles (01/2015 - 01/2006)
9.	Zhang, Wei: 8 articles (12/2014 - 06/2011)
10.	Zhou, Dao-Bin: 7 articles (10/2015 - 06/2011)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to POEMS Syndrome:

1.	lenalidomide (CC 5013)FDA Link 03/01/2013 - "Lenalidomide thus appears to be effective in POEMS syndrome, inducing high rate of clinical and biological responses." 03/01/2013 - "Efficacy of lenalidomide in POEMS syndrome: a retrospective study of 20 patients." 11/01/2012 - "Even though literature describes remission of POEMS syndrome following lenalidomide, four cycles did not lead to remission in our patient. " 06/01/2015 - "Lenalidomide as frontline therapy in polyneuropathy, organomegaly, endocrinopathy, monoclonal protein and skin changes syndrome: a retrospective case series of eight patients." 09/01/2014 - "The purpose of this pooled analysis was to synthesize all available data so as to evaluate the efficacy and safety of lenalidomide in patients with POEMS syndrome. "
2.	Dexamethasone (Maxidex)FDA LinkGeneric 07/01/1995 - "Combined high-dose 7S-IgG and dexamethasone is effective in severe polyneuropathy of the POEMS syndrome." 10/01/2015 - "In conclusion, low-dose lenalidomide plus dexamethasone therapy is an effective and safe regimen for patients with relapsed or refractory POEMS syndrome. " 10/01/2015 - "In this study, we used low-dose lenalidomide (10 mg daily) and dexamethasone (40 mg, once weekly) to treat twelve patients with relapsed (n = 8) or refractory (n = 4) POEMS syndrome. " 10/01/2015 - "Efficacy and safety of low-dose lenalidomide plus dexamethasone in patients with relapsed or refractory POEMS syndrome." 03/01/2013 - "We report the use of a combination of lenalidomide and dexamethasone in 20 patients with POEMS syndrome. "
3.	Thalidomide (Thalomid)FDA Link 01/01/2015 - "She achieved partial remission of WM, and thalidomide was continued for POEMS syndrome. " 01/01/2015 - "The Japanese POEMS syndrome with Thalidomide (J-POST) Trial is a phase II/III multicentre, double-blinded, randomised, controlled trial that aims to evaluate the efficacy and safety of a 24-week treatment with thalidomide in POEMS syndrome, with an additional 48-week open-label safety study. " 01/01/2015 - "Japanese POEMS syndrome with Thalidomide (J-POST) Trial: study protocol for a phase II/III multicentre, randomised, double-blind, placebo-controlled trial." 11/01/2008 - "To investigate the effects of thalidomide treatment for POEMS syndrome. " 08/01/2006 - "Thalidomide for POEMS syndrome."
4.	Vascular Endothelial Growth Factor A (Vascular Endothelial Growth Factor)IBA 01/01/2015 - "We tested the predictive efficacy of serum VEGF levels in POEMS syndrome. " 10/27/2011 - "Several studies have compared serum VEGF levels between POEMS patients and other disease entities showing higher serum VEGF in POEMS syndrome; however, it is unknown whether serum levels are reliable and reproducible given variable platelet release of VEGF. " 10/01/2015 - "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). " 10/01/2015 - "The significant correlation between the serum VEGF level and subfoveal CT in patients with POEMS syndrome suggests that choroidal thickness is influenced by the level of serum VEGF. " 10/01/2015 - "Correlation between serum level of vascular endothelial growth factor and subfoveal choroidal thickness in patients with POEMS syndrome."
5.	Melphalan (Alkeran)FDA LinkGeneric 07/01/2011 - "Melphalan-based therapy (OR, 0.076; 95% CI, 0.02-0.285) and normal renal function (OR, 0.246; 95% CI, 0.076-0.802) were independent prognostic factors for the survival of patients with POEMS syndrome. " 10/01/2004 - "High-dose melphalan with auto-PBSCT should be investigated further as a recommended therapy for POEMS syndrome." 09/01/2002 - "We report two cases of POEMS syndrome with solitary asymptomatic bone lesions treated with high-dose melphalan (200 mg/m(2)) and peripheral blood stem cell (PBSC) rescue. " 04/01/2006 - "We treated a 61-year-old woman with POEMS syndrome with high-dose melphalan therapy (HDT) supported by autologous blood stem cell transplantation (SCT), and clinical remission was achieved. " 01/01/2015 - "Autologous hematopoietic stem cell transplantation following high-dose melphalan is an effective therapy for younger patients with widespread osteosclerotic lesions in POEMS syndrome."
6.	bortezomib (Velcade)FDA Link 02/01/2010 - "Bortezomib may be an effective and safe therapeutic option for patients with POEMS syndrome." 12/01/2013 - "Bortezomib was well tolerated and we suggest that BorDex therapy followed by HDT and ASCT may be an effective therapy for POEMS syndrome. " 01/01/2013 - "Effective induction therapy with subcutaneous administration of bortezomib for newly diagnosed POEMS syndrome: a case report and a review of the literature." 02/01/2010 - "We present a 65-year-old man with POEMS syndrome who was successfully treated with bortezomib. " 02/01/2010 - "Bortezomib: a new therapeutic option for POEMS syndrome."
7.	Adrenal Cortex Hormones (Corticosteroids)IBA 02/01/2007 - "First-line therapy should include corticosteroids in POEMS syndrome-related PH." 08/01/2009 - "Serum levels of BNP dropped to 8 pg/mL. Our findings suggest that corticosteroids could play a role as a first-line treatment in pulmonary hypertension accompanied by POEMS syndrome. " 08/01/2009 - "Systemic corticosteroids as first-line treatment in pulmonary hypertension associated with POEMS syndrome." 08/01/2009 - "We present the case of a 54-year-old woman diagnosed with POEMS syndrome and pulmonary hypertension, which were treated with corticosteroids as the first-line therapy. " 01/01/1990 - "Some patients in whom POEMS syndrome was associated, or not, with myeloma were treated with chemotherapy and/or radiotherapy with different responses; in others, corticosteroids were of short lived benefit. "
8.	Cyclophosphamide (Cytoxan)FDA LinkGeneric 02/20/1999 - "In june 1993, a 58-year-old man was treated by cyclophosphamide and steroid for a POEMS syndrome. " 02/20/1999 - "This observation offers three original types of information: there has been no prior report of an association between veno-occlusive disease and POEMS syndrome, the role of low dose cyclophosphamide in hepatic veno-occlusive disease was certain, transjugular intrahepatic portosystemic shunt is an interesting therapy with one years remission." 10/01/2011 - "We have therefore reviewed our experience with ASCT in patients with POEMS syndrome who were treated with cyclophosphamide and prednisone as induction therapy followed by cyclophosphamide mobilization with an emphasis on treatment-related morbidity and frequency of ES. " 10/01/2011 - "Cyclophosphamide and prednisone induction followed by cyclophosphamide mobilization effectively decreases the incidence of engraftment syndrome in patients with POEMS syndrome who undergo stem cell transplantation." 11/01/2012 - "A patient with osteosclerotic myeloma and POEMS syndrome was initially treated with CAD chemotherapy (cyclophosphamide, adriamycine and dexamethazone) that was followed by tandem high dose chemotherapy (melphalan 100 mg/m2) and autologous transplantation. "
9.	Immunoglobulins (Immunoglobulin)IBA 08/01/2012 - "Several studies have demonstrated the restrict usage of immunoglobulin λ light chain variable region (IGLV) genes in patients with POEMS syndrome. " 07/01/2014 - "In summary, the overproduction of polyclonal immunoglobulin accounts for the high frequency of normal sFLC-R in patients with POEMS syndrome. " 07/01/2014 - "Serum immunoglobulin free light chain and heavy/light chain measurements in POEMS syndrome." 08/01/2012 - "Characterization of immunoglobulin λ light chain variable region (IGLV) gene and its relationship with clinical features in patients with POEMS syndrome." 06/01/2010 - "Serum immunoglobulin free light chain measurements and heavy chain isotype usage provide insight into disease biology in patients with POEMS syndrome."
10.	Multi-centric Castleman's DiseaseIBA 10/01/2011 - "Polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes syndrome is a rare multi-systematic disorder of uncertain etiology, if associated with multicentric Castleman's disease, it can lead to a more serious condition. " 10/01/2009 - "Glomeruloid haemangioma is considered a specific marker of POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes) syndrome and it is usually but not always associated with multicentric Castleman's disease. " 01/01/2007 - "Images in neuro-oncology: a case of POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal protein and Skin changes) in a patient with multicentric Castleman's disease." 07/01/2004 - "A transitional type of multicentric Castleman's disease may be present itself as POEMS syndrome. " 06/27/1998 - "We report on a 59-year-old male with a complete form of POEMS syndrome associated with multicentric Castleman's disease. "

Therapies and Procedures

1.	Peripheral Blood Stem Cell Transplantation (Peripheral Stem Cell Transplantation) 01/01/2015 - "Autologous peripheral blood stem cell transplantation (aPBSCT) is an effective treatment for POEMS syndrome. " 04/01/2013 - "Autologous peripheral blood stem cell transplantation is regarded as a useful treatment for POEMS syndrome." 01/01/2014 - "[Efficacy of autologous peripheral blood stem cell transplantation (auto-PBSCT) on the neuropathic manifestations in POEMS syndrome]." 04/01/2013 - "[A case of POEMS syndrome responding to autologous peripheral blood stem cell transplantation]." 02/01/2012 - "Usefulness of F-18 FDG PET/CT in the follow-up of POEMS syndrome after autologous peripheral blood stem cell transplantation."
2.	Drug Therapy (Chemotherapy) 08/01/2007 - "AutoPBSCT with high-dose chemotherapy should be considered among the treatments of choice for POEMS syndrome." 10/01/2006 - "We describe the clinical course and long term follow-up of a patient with POEMS syndrome unresponsive to standard chemotherapy. " 08/01/2006 - "Successful treatment of a patient with POEMS syndrome by tandem high-dose chemotherapy with autologous CD34+ purged stem cell rescue." 05/01/2013 - "Efficacy of high-dose chemotherapy with autologous peripheral blood stem cell transplantation for POEMS syndrome has been reported. " 03/01/2014 - "High-dose chemotherapy followed by autologous stem cell transplantation has shown encouraging efficacy in the treatment of patients with POEMS syndrome. "
3.	Stem Cell Transplantation 05/12/2015 - "To study the evolution of the neuropathy and long-term disability in a large cohort of patients with POEMS (polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes) syndrome following autologous stem cell transplantation (ASCT). " 01/01/2015 - "Pulmonary morbidity improves after autologous stem cell transplantation in POEMS syndrome." 02/01/2014 - "Autologous stem cell transplantation for POEMS syndrome." 11/01/2012 - "Long-term outcomes after autologous stem cell transplantation in patients with POEMS syndrome." 07/05/2012 - "Long-term outcomes after autologous stem cell transplantation for patients with POEMS syndrome (osteosclerotic myeloma): a single-center experience."
4.	Radiotherapy 01/01/1993 - "MRI examination was very useful for grasping the tumorous extent within the osteosclerotic lesion as observed with Crow-Fukase syndrome and for determining the extent of indication for surgery and radiotherapy." 01/01/2014 - "In this study, we examined the treatment outcomes and role of radiotherapy in the management of POEMS syndrome. " 01/01/2014 - "The role of radiotherapy in the management of POEMS syndrome." 02/01/2012 - "After autologous peripheral blood stem cell transplantation, F-18 FDG PET/CT may play a pivotal role in detecting new bone lesions in patients with POEMS syndrome, which may be treated by a focalized radiotherapy and/or systemic therapy." 06/01/2008 - "We reviewed the current treatment strategies for POEMS syndrome and summarized them as follows; 1) Radiotherapy or surgical resection is highly effective for isolated plasmacytoma. "
5.	Transplants (Transplant) 05/01/2008 - "Peripheral blood stem cell transplant for POEMS syndrome is associated with high rates of engraftment syndrome." 10/01/2006 - "Autologous peripheral stem cell transplant for POEMS syndrome: a case report." 06/01/2009 - "Two with polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes (POEMS) syndrome improved-improvement was sustained in one but relapsed and required repeat transplant in the other. " 01/01/2012 - "A diagnosis of POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes) with plasmacytoma was established; a post-transplant lymphoproliferative disorder was suspected." 01/01/2015 - "Patients with POEMS syndrome treated with aPBSCT have significant improvement in PFTs, respiratory muscle strength, imaging, and post-transplant IL-6. "