keyword
https://read.qxmd.com/read/37837287/small-vessel-platelet-thrombosis-in-the-peripheral-nerves-in-poems-syndrome
#21
JOURNAL ARTICLE
Hiroki Takeuchi, Yasumasa Yamamoto, Junichi Miyahara, Takao Takeshima, Akihiko Ozaki, Teruaki Kawasaki, Kazuyuki Kawamura, Nobuyuki Oka
INTRODUCTION/AIMS: Vascular thrombosis is prevalent among patients with polyneuropathy, organomegaly, endocrinopathy M-protein, and skin changes (POEMS) syndrome. The endothelial cells in the endoneurium are often hypertrophied and the lumen is frequently occluded. Consequent local hypoxia may increase vascular endothelial growth factor (VEGF), which induces hypercoagulation and vascular permeability. METHODS: This study presents two patients in the fifth decade of life, who had rare nerve biopsy findings of vascular occlusion mainly by platelets...
November 2023: Muscle & Nerve
https://read.qxmd.com/read/37793230/a-case-report-on-poems-syndrome-with-high-parathyroid-hormone-and-repeated-surgery-a-rare-presentation-of-a-rare-disease
#22
Aram Yaseri, Shima Ghannadi, Lida Perseh, Zahra Hoseini Tavassol, Shirin Hasani-Ranjbar
INTRODUCTION: Polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes (POEMS) syndrome is a rare multisystemic disease that is named for its constellation which can easily be mistaken for other disorders. CASE PRESENTATION: In present study we evaluated a 39-year-old man with hypercalcemia and parathyroid hormone. He underwent two failed surgeries for parathyroid adenoma but due to the presence of other features of the syndrome, a diagnosis of POEMS syndrome was made...
October 2023: International Journal of Surgery Case Reports
https://read.qxmd.com/read/37786834/castleman-disease-variant-of-poems-syndrome-complicated-with-multiple-osteosclerotic-lesions
#23
JOURNAL ARTICLE
Manaswinee Mallik, Lakshmon Kalikkaleth, Naresh Kumar Chirumamilla, Arihant Jain, Charanpreet Singh, Aditya Jandial, Amanjit Bal, Deepesh Lad, Alka Khadwal, Pankaj Malhotra, Gaurav Prakash
No abstract text is available yet for this article.
October 2023: Indian Journal of Hematology & Blood Transfusion
https://read.qxmd.com/read/37767406/baricitinib-treatment-for-refractory-skin-changes-in-poems-syndrome-a-case-report
#24
Jingjing Xie, Zhiling Li, Yubao Jiang, Dabin Tang, Xia Qiu, Ertao Jia, Jianyong Zhang
Polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, and skin changes (POEMS) syndrome is a multisystem disorder that has limited treatment options. Here, we described a case of a 55-year-old female subject who was treated for multiple drugs, but the skin symptoms continued to progress; the patient responded well to baricitinib. This suggests that JAK/STAT signaling pathways play an essential role in the pathological process of POEMS syndrome.
2023: Frontiers in Pharmacology
https://read.qxmd.com/read/37750557/a-case-of-atypical-igg4-related-disease-presenting-hypereosinophilia-polyneuropathy-and-liver-dysfunction
#25
JOURNAL ARTICLE
Hiroki Mukoyama, Kosaku Murakami, Hideo Onizawa, Mirei Shirakashi, Ryosuke Hiwa, Hideaki Tsuji, Koji Kitagori, Shuji Akizuki, Ran Nakashima, Akira Onishi, Hajime Yoshifuji, Masao Tanaka, Akio Morinobu
IgG4-related disease affects several organs, including the salivary glands, lacrimal glands, pancreas, biliary ducts, and retroperitoneum. A 72-year-old woman was examined for hypereosinophilia, high levels of IgG4, polyneuropathy, liver dysfunction, enlargement of lymph nodes and lacrimal glands, and beaded dilation of the bile ducts. We diagnosed IgG4-related disease based on biopsies of the lymph nodes, liver, and submandibular gland. The symptoms of the patient improved after glucocorticoid treatment. This was a novel and atypical case of IgG4-related disease that was difficult to differentiate from other diseases, including eosinophilic granulomatosis with polyangiitis, idiopathic hypereosinophilic syndrome, and POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal plasma cell disorder, Skin changes) syndrome...
September 26, 2023: Modern rheumatology case reports
https://read.qxmd.com/read/37732822/poems-syndrome-update-on-diagnosis-risk-stratification-and-management
#26
JOURNAL ARTICLE
Angela Dispenzieri
DISEASE OVERVIEW: POEMS syndrome is a life-threatening condition due to an underlying plasma cell neoplasm. The major criteria for the syndrome are polyradiculoneuropathy, clonal plasma cell disorder, 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. 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...
September 21, 2023: American Journal of Hematology
https://read.qxmd.com/read/37714363/a-rare-cause-of-chronic-diarrhea-and-peripheral-neuropathy
#27
JOURNAL ARTICLE
Hansol Kang, Maimon E Hubbi
No abstract text is available yet for this article.
September 14, 2023: Gastroenterology
https://read.qxmd.com/read/37702830/regular-assessment-of-serum-vascular-endothelial-growth-factor-levels-to-monitor-poems-syndrome
#28
JOURNAL ARTICLE
Francesco Gentile, Fabrizia Terenghi, Pietro Emiliano Doneddu, Alberto De Lorenzo, Claudia Giannotta, Andrea Giordano, Rita Mazza, Andrea Nozza, Eduardo Nobile-Orazio
BACKGROUND: To investigate the utility of regular serum VEGF (sVEGF) levels assessment in the monitoring of POEMS syndrome. METHODS: We retrospectively reviewed data of 30 patients with POEMS syndrome whose sVEGF was tested regularly every 6 months. sVEGF levels after treatment were measured and correlated with disability (Overall Neuropathy Limitations Scale, ONLS), clinical impairment (measured with the modified Clinical Response Evaluation Scale, mCRES), and relapse-free survival...
September 13, 2023: Neurological Sciences
https://read.qxmd.com/read/37699737/poems-syndrome-an-unusual-cause-of-exuberant-callus-formation
#29
JOURNAL ARTICLE
Bhanu Malhotra, Ashwini Sood, Pankaj Malhotra, Sanjay Bhadada
Several growth factors are expressed in distinct temporal and spatial patterns during fracture repair. Together, they lead to angiogenesis-osteogenesis coupling and hence bone healing by callus formation. Of these growth factors, vascular endothelial growth factor is of particular interest because of its ability to induce neovascularisation (angiogenesis) and osteoclast invasion in soft callus. Hyperplastic callus can be seen in metabolic bone disease, endocrine disease and in patients with traumatic brain injury, but has not been described so far in polyneuropathy, organomegaly, endocrinopathy, M-protein, skin changes (POEMS) syndrome...
September 12, 2023: BMJ Case Reports
https://read.qxmd.com/read/37646655/which-is-the-best-treatment-strategy-before-autologous-peripheral-blood-stem-cell-transplantation-in-poems-syndrome
#30
JOURNAL ARTICLE
Francesco Autore, Stefania Bramanti, Federica Lessi, Idanna Innocenti, Eugenio Galli, Serena Rocchi, Rossella Ribolla, Daniele Derudas, Stefania Oliva, Paola Stefanoni, Magda Marcatti, Angelo Schenone, Giorgio La Nasa, Claudia Crippa, Elena Zamagni, Marcello Riva, Rita Mazza, Daniele Mannina, Simona Sica, Andrea Bacigalupo, Luca Laurenti
Autologous peripheral blood stem cell transplantation (aPBSCT) provides optimal outcomes in POEMS syndrome but the definition of the best treatment before aPBSCT remains to be defined, because of the disease rarity and the heterogeneity of published case series. We collected clinical and laboratory data of patients with POEMS syndrome undergoing aPBSCT from 1998 to 2020 in 10 Italian centres. The primary endpoint of the study was to evaluate the impact of prior therapies and mobilizing regimen on outcome. We divided patients in three groups: patients who did not receive any treatment before transplant (15 patients, group A: front-line), pre-treated patients with other agents (14 patients, group B) and patients treated with cyclophosphamide as mobilizing regimen (16 patients, group C)...
August 31, 2023: Haematologica
https://read.qxmd.com/read/37573150/characteristics-and-outcome-of-infectious-complications-after-autologous-hematopoietic-cell-transplantation-in-multiple-myeloma-patients
#31
JOURNAL ARTICLE
Aditya Jandial, Deepesh Lad, Arihant Jain, Alka Khadwal, Charanpreet Singh, Gaurav Prakash, Vikas Suri, Sreejesh Sreedharanunni, Man Updesh Singh Sachdeva, Pallab Ray, Neelam Varma, Subhash Varma, Pankaj Malhotra
BACKGROUND: Infections are a significant cause of morbidity and mortality after autologous hematopoietic cell transplantation (AHCT) in multiple myeloma (MM) patients. There has been a rapid advancement and evolution in MM treatment landscape in the last decade. There is limited information on post-AHCT infectious complications among MM patients with or without levofloxacin prophylaxis from developing countries. MATERIALS AND METHODS: We performed a retrospective study to explore the incidence, pattern, and clinical outcome of infections following AHCT in MM patients from 2010 to 2019 at our center...
August 12, 2023: Transplant Infectious Disease: An Official Journal of the Transplantation Society
https://read.qxmd.com/read/37560829/-poems-syndrome
#32
JOURNAL ARTICLE
I E Arkhipov, I Yu Vergunova, N A Malkova, D S Korobko
POEMS - syndrome (polyneuropathy - P, organomegaly - O, endocrinopathy - E, M-protein - M, skin changes - S) is a paraneoplastic syndrome caused by underlying dyscrasia of plasma cells. The main criteria of the syndrome are polyradiculoneuropathy, clonal proliferation of plasma cells, sclerotic bone lesions, elevated levels of vascular endothelial growth factor and the presence of Castleman disease. Additional signs include organomegaly, endocrinopathy, characteristic skin changes, edema of the optic disc, extravascular volume overload (edema) and thrombocytosis...
2023: Zhurnal Nevrologii i Psikhiatrii Imeni S.S. Korsakova
https://read.qxmd.com/read/37544716/-identification-of-transcriptional-features-and-surface-markers-of-plasma-cell-clones-in-poems-syndrome-by-single-cell-rna-sequencing
#33
JOURNAL ARTICLE
Yusuke Isshiki, Motohiko Oshima, Naoya Mimura, Emiko Sakaida, Atsushi Iwama
POEMS syndrome is a rare monoclonal plasma cell disorder with unique symptoms distinct from other plasma cell neoplasms. To identify and find the transcriptional features of clonal plasma cells in POEMS syndrome (POEMS clones), single-cell RNA sequencing was performed on patient-derived bone marrow plasma cells. POEMS clones were identified in 5 out of 10 patients, and the proportions of POEMS clones in the plasma cells were markedly smaller than that of other plasma cell malignancies such as multiple myeloma and MGUS...
2023: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://read.qxmd.com/read/37530355/poems-syndrome-a-rare-entity
#34
Chaganti P Devi, Basimalla R Stephenson, P Ramana Kumari, Panchakarla G Vani, Swargam P Madhooli
POEMS syndrome is a rare paraneoplastic syndrome due to an underlying plasma cell disorder. The diagnosis of POEMS syndrome can be a challenge. A good history, physical examination, and appropriate testing can aid in establishing its diagnosis. We are presenting the case of a 75-year-old man who was diagnosed with POEMS syndrome.
2023: Indian Journal of Pathology & Microbiology
https://read.qxmd.com/read/37522432/treatment-responsive-glycogen-storage-myopathy-in-a-patient-with-poems-syndrome-a-new-monoclonal-gammopathy-associated-myopathy
#35
Pannathat Soontrapa, Jennifer A Tracy, Wilson I Gonsalves, Teerin Liewluck
BACKGROUND: Myopathies associated with monoclonal gammopathy are relatively uncommon and underrecognized, treatable myopathies, and include sporadic late onset nemaline myopathy, light chain amyloid myopathy, and a recently described vacuolar myopathy with monoclonal gammopathy and stiffness (VAMGS). Herein, we report a new subtype of monoclonal gammopathy-associated myopathy (MGAM) in a polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes (POEMS) patient. METHOD: Case report...
October 2023: European Journal of Neurology
https://read.qxmd.com/read/37503535/advances-in-the-diagnosis-and-management-of-achalasia-and-achalasia-like-syndromes-insights-from-hrm-and-flip
#36
JOURNAL ARTICLE
Peter J Kahrilas, Dustin A Carlson, John E Pandolfino
High-resolution manometry, Chicago Classification v4.0, the functional lumen imaging probe, Panometry, and peroral endoscopic myotomy (POEM) are all now integral parts of the landscape for managing achalasia or, more precisely, achalasia-like syndromes. This narrative review examines the impact of these innovations on the management of achalasia-like syndromes. High-resolution manometry was the disruptive technology that prompted the paradigm shift to thinking of motility disorders as patterns of obstructive physiology involving the esophagogastric junction and/or the distal esophagus rather than as siloed entities...
2023: Gastro Hep Adv
https://read.qxmd.com/read/37391497/alterations-in-choroidal-vascular-structures-due-to-serum-levels-of-vascular-endothelial-growth-factor-in-patients-with-poems-syndrome
#37
JOURNAL ARTICLE
Hirotaka Yokouchi, Daisuke Nagasato, Yoshinori Mitamura, Mariko Egawa, Hitoshi Tabuchi, Sonoko Misawa, Satoshi Kuwabara, Takayuki Baba
A higher serum vascular endothelial growth factor (VEGF) level can cause choroidal thickening in the choroid of patients with polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome. We aimed to determine whether fluctuations in serum VEGF levels affect choroidal vascular structures in patients with POEMS syndrome. This retrospective observational case series examined 17 left eyes of 17 patients with POEMS syndrome. Enhanced depth imaging optical coherence tomography (EDI-OCT) images were obtained, and serum VEGF levels were measured at baseline and 6 months after transplantation with dexamethasone (n = 6), thalidomide (n = 8), or lenalidomide (n = 3)...
June 30, 2023: Scientific Reports
https://read.qxmd.com/read/37376758/ultrasound-based-radiomic-analysis-of-the-peripheral-nerves-for-differentiation-between-cidp-and-poems-syndrome
#38
JOURNAL ARTICLE
Jun Hashiba, Hajime Yokota, Kota Abe, Yukari Sekiguchi, Shinobu Ikeda, Atsuhiko Sugiyama, Satoshi Kuwabara, Takashi Uno
BACKGROUND: Demyelinating peripheral neuropathy is characteristic of both polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes (POEMS) syndrome and chronic inflammatory demyelinating polyneuropathy (CIDP). We hypothesized that the different pathogeneses underlying these entities would affect the sonographic imaging features. PURPOSE: To investigate whether ultrasound (US)-based radiomic analysis could extract features to describe the differences between CIDP and POEMS syndrome...
June 27, 2023: Acta Radiologica
https://read.qxmd.com/read/37341332/hematologic-disorders-and-the-nervous-system
#39
JOURNAL ARTICLE
Michelle L Mauermann, Andrew M Southerland
OBJECTIVE: This article discusses the epidemiology, diagnosis, treatment, and prevention of neurologic complications of red blood cell, platelet, and plasma cell disorders. LATEST DEVELOPMENTS: Cerebrovascular complications can occur in patients with blood cell and platelet disorders. Treatment strategies to prevent stroke are available for patients with sickle cell disease, polycythemia vera, and essential thrombocythemia. A diagnosis of thrombotic thrombocytopenic purpura should be considered in patients with neurologic symptoms, hemolytic anemia, thrombocytopenia, mild renal insufficiency, and fever...
June 1, 2023: Continuum: Lifelong Learning in Neurology
https://read.qxmd.com/read/37228016/targeted-testing-of-bone-marrow-specimens-with-cytoplasmic-vacuolization-to-identify-previously-undiagnosed-cases-of-vexas-syndrome
#40
JOURNAL ARTICLE
Alexander S Hines, Matthew J Koster, Allison R Bock, Ronald S Go, Kenneth J Warrington, Horatiu Olteanu, Terra L Lasho, Mrinal M Patnaik, Kaaren K Reichard
OBJECTIVE: To retrospectively identify patients with VEXAS syndrome (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic syndrome) among male patients with bone marrow vacuolization using a clinically applicable, targeted-screening approach. METHODS: Bone marrow reports from 1 May 2014 through 18 Feb 2022 were reviewed for documentation of cytoplasmic vacuolization. Patients with acute leukaemia, lymphoma, metastatic solid tumor, amyloidosis, or POEMS were excluded as were those without clinical records available for direct chart review...
May 25, 2023: Rheumatology
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