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https://www.readbyqxmd.com/read/29179528/ischemic-stroke-in-patients-with-poems-syndrome-a-case-report-and-comprehensive-analysis-of-literature
#1
Fang-Wang Fu, Jie Rao, Yuan-Yuan Zheng, Hui-Lin Wang, Jian-Guang Yang, Guo-Qing Zheng
Background: POEMS syndrome is a rare multi-systemic disease characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes. Arterial or venous thrombosis is a less-common complication of POEMS syndrome. Ischemic stroke has also been reported sporadically. However, the association between POEMS syndrome and ischemic stroke has not been entirely understood. Methods: A case of ischemic stroke caused by cerebral vasculitis in a patient with POEMS syndrome was presented...
October 24, 2017: Oncotarget
https://www.readbyqxmd.com/read/29157619/diagnosis-of-castleman-disease
#2
REVIEW
Raphaël Szalat, Nikhil C Munshi
Castleman disease (CD) is a rare and heterogenous group of disorders sharing in common an abnormal lymph node pathology. CD comprises distinct subtypes with different prognoses. Unicentric CD and multicentric CD are featured by specific systemic manifestations and may be associated with Kaposi sarcoma, non-Hodgkin and Hodgkin lymphoma, and POEMS syndrome. Multicentric CD is classically associated with systemic symptoms and poorer prognosis. In this article, the authors review how to diagnose the disease, keeping in context the clinical findings, biochemical changes and complications associated with CD...
February 2018: Hematology/oncology Clinics of North America
https://www.readbyqxmd.com/read/29157616/the-peripheral-neuropathies-of-poems-syndrome-and-castleman-disease
#3
REVIEW
Michelle L Mauermann
Polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, skin changes (POEMS) syndrome is a rare paraneoplastic disorder. The polyneuropathy can be the presenting symptom and is typically a painful, motor-predominant polyradiculoneuropathy often mimicking chronic inflammatory demyelinating polyradiculoneuropathy. The presence of a lambda monoclonal protein, elevated vascular endothelial growth factor, systemic features, and treatment resistance are clues to the diagnosis. Castleman disease (CD) is seen in a subset of these patients, and when present the neuropathy is similar but less severe...
February 2018: Hematology/oncology Clinics of North America
https://www.readbyqxmd.com/read/29157615/poems-syndrome-therapeutic-options
#4
REVIEW
Arnaud Jaccard
Treatment of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome should be directed at the underlying plasma cell clone with risk-adapted therapy based on the extent of the plasma cell disorder. Radiation therapy is effective for patients with a localized presentation, without bone marrow involvement, and 1 to 3 bone lesions. Patients with disseminated disease should receive, preferably, high-dose chemotherapy with peripheral blood transplantation. Low-dose melphalan and dexamethasone or new agents used in myeloma are also effective...
February 2018: Hematology/oncology Clinics of North America
https://www.readbyqxmd.com/read/29157614/poems-syndrome-diagnosis-and-investigative-work-up
#5
REVIEW
Angela Dispenzieri, Taxiarchis Kourelis, Francis Buadi
POEMS syndrome is a rare paraneoplastic syndrome secondary to a plasma cell dyscrasia. Recognition of a combination of peripheral neuropathy, organomegaly, endocrinopathy, monoclonal plasmaproliferative disorder, skin changes, papilledema, extravascular volume overload, sclerotic bone lesions, thrombocytosis, and Castleman disease is the first step in managing the disease. Increased blood levels of vascular endothelial growth factor are usually confirmatory. This rare disorder should not be missed, especially if the patient has a putative diagnosis of chronic inflammatory polyradiculoneuropathy, a lambda restricted monoclonal gammopathy, and thrombocytosis, and is not responding as expected to immunomodulatory therapy commonly used for chronic inflammatory polyradiculoneuropathy...
February 2018: Hematology/oncology Clinics of North America
https://www.readbyqxmd.com/read/29113155/poems-syndrome-presentation-with-progressive-weakness-in-upper-and-lower-limbs-a-case-report
#6
Chuang-Jie Cao, Cheng-Yun Dou, Ke-Hua Zhou, Jin-Bo Liu, Hong Lai
Polyneuropathy, organomegaly, endocrinopathy, M proteins, and skin changes (POEMS) syndrome is a rare variant of plasma cell disorders with multiple systemic manifestations. A 50-year-old female patient presented with progressive weakness in her upper and lower limbs; tingling, numbness and burning in her feet; polyneuropathy (demyelinating in the majority of cases of POEMS syndrome); monoclonal plasma cell disorder (typicallyλ-restricted in cases of POEMS syndrome); sclerotic lesions on the spine and pelvis; organomegaly, including hepatomegaly, splenomegaly and lymphadenopathy; edema; pleural effusion; adrenal, thyroidal, pituitary, gonadal and pancreatic endocrinopathy; skin changes, including hyperpigmentation, dry skin and hypertrichosis; thrombocytosis; pulmonary hypertension; low vitamin B12 and weight loss...
November 2017: Oncology Letters
https://www.readbyqxmd.com/read/29100675/skull-base-plasmacytoma-a-unique-case-of-poems-syndrome-with-a-plasmacytoma-causing-craniocervical-instability
#7
Hannah Gilder, Meghan E Murphy, Mohammed Ali Alvi, Panagiotis Kerezoudis, Daniel Shepherd, Patrick R Maloney, Michael J Yaszemski, Jonathan M Morris, Angela Dispenzieri, Jane M Matsumoto, Mohamad Bydon
INTRODUCTION: Plasmacytomas, considered to be the solitary counterparts of multiple myeloma, are neoplastic monoclonal plasma cell proliferations within soft tissue or bone. Plasmacytomas often present as a collection of findings known as POEMS-syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, M-Protein spike, and Skin changes). CASE DESCRIPTION: We present a report of a 47 yo male diagnosed with POEMS-syndrome secondary to a skull base plasmacytoma. The mass resulted in marked instability of the cranio-cervical junction due to bony erosion...
October 31, 2017: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
https://www.readbyqxmd.com/read/29056130/an-adult-with-polyneuropathy-and-hypogonadism-due-to-poems-syndrome
#8
Saba Zaidi, Sidra Sattar, Khealani Bhojo Asumal
POEMS (acronym for polyneuropathy, organomegaly, endocrinopathy, M protein myeloma and skin changes), is a rare disease which occurs in the setting of plasma cell dyscrasias. We describe a case of an adult lady who presented with gradual onset weakness of all four limbs and multisystem involvement characterized by pedal edema, ascites, hyperpigmentation and hypogonadism. Nerve conduction study showed severe sensorimotor polyneuropathy. Serum immunofixation showed lambda light chain restricted monoclonal gammopathy...
October 2017: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
https://www.readbyqxmd.com/read/29048107/lenalidomide-and-dexamethasone-in-patients-with-poems-syndrome-results-of-a-prospective-open-label-trial
#9
Andrea Nozza, Fabrizia Terenghi, Francesca Gallia, Fausto Adami, Chiara Briani, Giampaolo Merlini, Laura Giordano, Armando Santoro, Eduardo Nobile-Orazio
Given its anti-angiogenic activity, lenalidomide may have a role in the treatment of POEMS (Peripheral neuropathy, Organomegaly, Endocrinopathy, Monoclonal plasma cell disorder and Skin changes) syndrome. This prospective, open-label, pilot study evaluated the combination of lenalidomide + dexamethasone (RD) in 18 POEMS syndrome patients (13 pre-treated, 5 newly-diagnosed but ineligible for high-dose therapy). Treatment consisted of six cycles of lenalidomide (25 mg/day for 21 days followed by 7 days rest) plus dexamethasone (40 mg/once a week)...
October 19, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/29034435/advances-in-the-treatment-of-paraproteinemic-neuropathy
#10
REVIEW
Eduardo Nobile-Orazio, Mariangela Bianco, Andrea Nozza
Purpose of review Several advances have been made on the pathogenesis and therapy of neuropathies associated with paraproteinemia (monoclonal gammopathy). It is important for the neurologist to understand the pathogenetic relevance of this association especially when the hematological disease does not require per se any therapy. Recent findings Treatment of the neuropathy in patients with malignant paraproteinemia is mainly addressed by the hematologist while the neurologist is mainly involved in the initial diagnosis and in deciding whether the neuropathy is caused by the disease or by the chemotherapy used for the disease...
October 16, 2017: Current Treatment Options in Neurology
https://www.readbyqxmd.com/read/29019884/utility-of-osteosclerotic-lesion-biopsy-in-diagnosis-of-poems-syndrome-a-case-report
#11
Daisuke Hara, Hisanao Akiyama, Saki Nukui, Takahiro Shimizu, Masahiro Hoshikawa, Yasuhiro Hasegawa
RATIONALE: We report a case of successful diagnosis of POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) syndrome based on monoclonality that was confirmed by an osteosclerotic lesion biopsy in a patient without pathognomonic symptoms or monoclonal gammopathy, probably because of comorbidities, which included systemic lupus erythematosus, rheumatoid arthritis, and Sjögren syndrome. PATIENT CONCERNS: A 57-year-old woman presented with an approximately 2-year history of numbness in the toes that had gradually spread, along with muscle weakness in both arms and legs...
October 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28989059/clinical-practice-update-the-use-of-per-oral-endoscopic-myotomy-in-achalasia-expert-review-and-best-practice-advice%C3%A2-from-the-aga-institute
#12
REVIEW
Peter J Kahrilas, David Katzka, Joel E Richter
The purpose of this review is to describe a place for per-oral endoscopic myotomy (POEM) among the currently available robust treatments for achalasia. The recommendations outlined in this review are based on expert opinion and on relevant publications from PubMed and EMbase. The Clinical Practice Updates Committee of the American Gastroenterological Association proposes the following recommendations: 1) in determining the need for achalasia therapy, patient-specific parameters (Chicago Classification subtype, comorbidities, early vs late disease, primary or secondary causes) should be considered along with published efficacy data; 2) given the complexity of this procedure, POEM should be performed by experienced physicians in high-volume centers because an estimated 20-40 procedures are needed to achieve competence; 3) if the expertise is available, POEM should be considered as primary therapy for type III achalasia; 4) if the expertise is available, POEM should be considered as treatment option comparable with laparoscopic Heller myotomy for any of the achalasia syndromes; and 5) post-POEM patients should be considered high risk to develop reflux esophagitis and advised of the management considerations (potential indefinite proton pump inhibitor therapy and/or surveillance endoscopy) of this before undergoing the procedure...
November 2017: Gastroenterology
https://www.readbyqxmd.com/read/28951579/expert-consensus-document-advances-in-the-management-of-oesophageal-motility-disorders-in-the-era-of-high-resolution-manometry-a-focus-on-achalasia-syndromes
#13
REVIEW
Peter J Kahrilas, Albert J Bredenoord, Mark Fox, C Prakash Gyawali, Sabine Roman, André J P M Smout, John E Pandolfino
High-resolution manometry (HRM) and new analysis algorithms, summarized in the Chicago Classification, have led to a restructured classification of oesophageal motility disorders. This advance has led to increased detection of clinically relevant disorders, in particular achalasia. It has become apparent that the cardinal feature of achalasia - impaired lower oesophageal sphincter (LES) relaxation - can occur in several disease phenotypes: without peristalsis (type I), with pan-oesophageal pressurization (type II), with premature (spastic) distal oesophageal contractions (type III), or with preserved peristalsis (outlet obstruction)...
November 2017: Nature Reviews. Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/28946631/alcohol-misuse-link-to-poems-syndrome-in-a-patient
#14
John Neary, Susan E Goodwin, Lawrence B Cohen, Manuela G Neuman
Previously called Crow-Fukase syndrome, POEMS syndrome is characterized by poly-neuropathy, osteo-sclerotic myeloma, organomegaly, endocrinopathy, monoclonal plasma cell disorder, and skin changes. Extremely elevated levels of serum vascular endothelial growth factor (VEGF) are characteristic of the syndrome. Chronic hepatitis B (HBV) and C (HCV) infections can also be present in POEMS. The pathogenesis of the syndrome is not well understood. The link between chronic alcohol consumption and this malignant condition has not been reported until now...
September 23, 2017: Cancers
https://www.readbyqxmd.com/read/28913957/autologous-peripheral-blood-stem-cell-transplantation-and-the-role-of-lenalidomide-in-patients-affected-by-poems-syndrome
#15
REVIEW
Francesco Autore, Idanna Innocenti, Marco Luigetti, Nicola Piccirillo, Federica Sora, Patrizia Chiusolo, Simona Sica, Andrea Bacigalupo, Luca Laurenti
POEMS syndrome is a rare paraneoplastic condition, with a poorly understood pathogenesis. High dose chemotherapy followed by autologous stem cell transplantation (ASCT) has been reported to be an effective therapeutic option for patients with good performance status. Here, we review the role of ASCT for POEMS syndrome and discuss indications together with advantages and disadvantages, and related issues such lenalidomide given before or after ASCT, VEGF levels as a marker of disease, and different regimens for stem cell mobilization...
September 15, 2017: Hematological Oncology
https://www.readbyqxmd.com/read/28906499/-incomplete-poems-syndrome-with-multicentric-castleman-s-disease
#16
P González de la Aleja, M García-Navarro, R Sánchez-Rodríguez, J M Ramos-Rincón
Castleman's disease (CD) is an atypical lymphoproliferative disorder of unknown cause, characterized by non-clonal nodal hyperplastic growth. Two forms of clinical presentation are currently recognized, one localized and the other multicentric, and four histopathologic variants. It is characterized by generalized lymphadenopathy, hepatosplenomegaly, fever and night sweats. CD may present severe pancytopenia, multi-organ failure, lymphoma evolution and it can sometimes be associated with paraneoplastic syndromes such as POEMS syndrome...
September 14, 2017: Anales del Sistema Sanitario de Navarra
https://www.readbyqxmd.com/read/28894560/poems-syndrome-an-update
#17
REVIEW
Andrea Nozza
POEMS syndrome is a rare, chronic and disabling condition. The causes of this condition remain unknown; however, chronic overproduction of proinflammatory cytokines appears to be a major contributor. Early diagnosis is essential to start treatment before the clinical state of the patient becomes compromised. A complete evaluation of the disease at its onset is critical to the treatment decision. In localized disease, curative doses of radiation (50 Gy) is the recommended therapy. On the other hand, patients with disseminated disease should be given systemic therapy...
2017: Mediterranean Journal of Hematology and Infectious Diseases
https://www.readbyqxmd.com/read/28893029/poems-syndrome-with-biclonal-gammopathy-a-rare-association
#18
Dibyendu De, Sandeepan Halder, Suvro Sankha Datta
Polyneuropathy, Organomegaly, Endocrinopathy, M protein and Skin changes (POEMS) syndrome is rare plasma cell dyscrasia with multisystem involvement. The name comes from the five characteristic features: Polyneuropathy, organomegaly, endocrinopathy, M protein and skin changes. The presence of biclonal M band is a rare manifestation. Here, we are describing the cases of a 60-year-old lady, presented with bilateral pedal oedema and pericardial effusion and peripheral neuropathy. She also had hepatosplenomegaly, hyperpigmented rash and hypothyroidism and hyperparathyroidism...
July 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28856089/plexogenic-pulmonary-hypertension-associated-with-poems-syndrome
#19
Thomas Czeczok, Peter Lin, Eunhee Yi
Pulmonary hypertension is one of the well-known clinical manifestations of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome, occurring in approximately 25-30% of the affected individuals. However, the histopathologic spectrum of pulmonary hypertension associated with POEMS syndrome has not been fully documented in the literature. Herein, we report an autopsy case of POEMS syndrome in a patient whose lung tissues showed histopathology indistinguishable from that of idiopathic pulmonary arterial hypertension with abundant plexiform lesions in the small pulmonary arteries...
2017: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/28833364/eosinophilia-secondary-to-lenalidomide-therapy
#20
V Escudero-Vilaplana, S Osorio-Prendes, R Collado-Borrell, E González-Arias, M Sanjurjo-Sáez
WHAT IS KNOWN AND OBJECTIVE: Limited data are available on eosinophilia as a drug adverse event. We describe a case of eosinophilia from lenalidomide therapy. CASE DESCRIPTION: A 50-year-old woman received lenalidomide, dexamethasone and cyclophosphamide as POEMS syndrome treatment. Eosinophil count rose during lenalidomide treatment and decreased in the periods off treatment. Naranjo nomogram suggested a probable association between the use of lenalidomide and eosinophilia...
August 17, 2017: Journal of Clinical Pharmacy and Therapeutics
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