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poems syndrome

Ting-Ting Han, Shuang Zheng, Zeng-Ai Chen, Wei Liu, Yao-Min Hu
POEMS syndrome is a rare multi-systemic disease characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal protein and skin changes. Arterial thrombosis is a distinctively unusual feature in patients with POEMS syndrome. We report a 33-year-old man with intermittent amaurosis of left eye and skin changes as the onset manifestations, who was finally confirmed as having POEMS syndrome. Most notably, this was a young man without high risk factors of arterial thrombosis and no monoclonal protein was detected until the repeated measurement later...
October 11, 2016: Oncotarget
Julio González-Martin-Moro, Inés Contreras, Belén Pilo-de-la-Fuente, Fernando Gómez-Sanz
PURPOSE: To report the correlation between visual function and subfoveal choroidal thickness (SChT) in a case of POEMS syndrome. CASE REPORT: A 53 year old man diagnosed with POEMS syndrome was referred due to blurred vision. Best corrected visual acuity (BCVA) was 0.5 in his right eye (RE) and 0.7 in his left eye (LE), with a mild perimetric defect in the RE. SChT was 356 and 263 µm in his RE and LE. After an autologous peripheral blood stem-cell transplantation, both visual and systemic symptoms improved...
October 11, 2016: Ocular Immunology and Inflammation
Hirotaka Yokouchi, Takayuki Baba, Sonoko Misawa, Masayasu Kitahashi, Toshiyuki Oshitari, Satoshi Kuwabara, Shuichi Yamamoto
AIMS: To determine the changes in the subfoveal choroidal thickness (CT), the foveal thickness (FT) and the serum level of vascular endothelial growth factor (VEGF) after thalidomide treatment in patients with polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes (POEMS) syndrome. METHODS: We studied 13 left eyes of 13 treatment-naïve patients with POEMS syndrome. The subfoveal CT and FT were determined by enhanced depth imaging optical coherence tomography, and the serum level of VEGF was determined by ELISA at the baseline and at 6 months after thalidomide treatment...
October 4, 2016: British Journal of Ophthalmology
Chen Wang, Xu-Fei Huang, Qian-Qian Cai, Xin-Xin Cao, Hao Cai, Daobin Zhou, Jian Li
Vascular endothelial growth factor (VEGF) is pathognomonically elevated in patients with POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes) syndrome. However, its source of overproduction is unclear. As clinical improvement is almost always associated with VEGF reduction after anti-plasma cell therapy, its increase at diagnosis has been attributed to the underlying monoclonal gammopathy, although direct evidence is still lacking. In the current study, we systemically measured VEGF levels in POEMS patients, before and after treatment...
September 26, 2016: Leukemia Research
Olivier Espitia, Jerome Connault, Mohamed A Hamidou
No abstract text is available yet for this article.
September 21, 2016: British Journal of Haematology
Gordon Cook, Simona Iacobelli, Anja van Biezen, Dimitris Ziagkos, Veronique LeBlond, Julie Abraham, Grant McQuaker, Stefan Schoenland, Alessandro Rambaldi, Kazimierz Halaburda, Maria Rovira, Simona Sica, Jenny Byrne, Ramon Garcia Sanz, Arnon Nagler, Niels W C J van de Donk, Marjatta Sinisalo, Mark Cook, Nicolaus Kröger, Theo De Witte, Curly Morris, Laurent Garderet
POEMS syndrome is a rare para-neoplastic syndrome secondary to a plasma cell dyscrasia. Effective treatment can control the disease-related symptom complex. We describe the clinical outcome of autologous stem cell transplantation for patients with POEMS syndrome, determining the impact of patient and disease-specific factors on prognosis. 127 patients underwent an autologous stem cell transplantation between 1997-2010 with a median age of 50 years (range 26-69). The median time from diagnosis to ASCT was 7...
September 15, 2016: Haematologica
Benedetta Miglino, Michele Viana, Rossana Tiberio, Paolo Boggio, Guido Valente, Gionata Strigaro, Enrico Colombo
The authors report a case of sensorimotor polyneuropathy, diffuse hemangiomas and monoclonal gammopathy. Besides weight loss, there were diabetes mellitus and severe hypothyroidism. These alterations were consistent with POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy and Skin changes) syndrome, which is a rare systemic disease with monoclonal proliferation of plasmacytes and slow progression. Because of its rarity, the incidence of this disease is still unknown.
September 15, 2016: Giornale Italiano di Dermatologia e Venereologia: Organo Ufficiale, Società Italiana di Dermatologia e Sifilografia
Fengdan Wang, Xufei Huang, Yan Zhang, Jian Li, Daobin Zhou, Zhengyu Jin
Objective. Bone lesion is crucial for diagnosing and management of polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin change (POEMS) syndrome, a rare plasma cell disorder. This study is to compare the effectiveness of X-ray skeletal survey (SS) and computed tomography (CT) for detecting bone lesions in Chinese POEMS syndrome patients, and to investigate the relationship between bone lesion features and serum markers. Methods. SS and chest/abdomen/pelvic CT images of 38 Chinese patients (26 males, 12 females, aged 21-70 years) with POEMS syndrome recruited at our medical center between January 2013 and January 2015 were retrospectively analyzed...
2016: PeerJ
F Rongioletti, M C Failla, L Atzori, C Ferreli
POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal protein and Skin signs) and AESOP (Adenopathy and Extensive Skin patch Overlying a Plasmacytoma) syndromes are rare paraneoplastic conditions due to an underlying plasma cell dyscrasia. We report a 70-year-old patient with the rare coexistence of POEMS and AESOP syndromes and in whom skin signs, that differ both clinically and histologically, were the clues to the diagnosis of a plasma cell disorder. Vascular endothelial growth factor-A overexpression seems to be the common pathogenetic link of the different clinico-pathological presentations of the skin lesions...
August 17, 2016: Journal of Cutaneous Pathology
Sonoko Misawa, Yasunori Sato, Kanako Katayama, Kengo Nagashima, Reiko Aoyagi, Yukari Sekiguchi, Gen Sobue, Haruki Koike, Ichiro Yabe, Hidenao Sasaki, Osamu Watanabe, Hiroshi Takashima, Masatoyo Nishizawa, Izumi Kawachi, Susumu Kusunoki, Yoshiyuki Mitsui, Seiji Kikuchi, Ichiro Nakashima, Shu-Ichi Ikeda, Nobuo Kohara, Takashi Kanda, Jun-Ichi Kira, Hideki Hanaoka, Satoshi Kuwabara
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...
October 2016: Lancet Neurology
Arnaud Jaccard, Laurent Magy
No abstract text is available yet for this article.
October 2016: Lancet Neurology
Eun Jung Kim, Dong Ho Shin, Hee Jung Jeon, So Yon Rhee, Eun Sook Nam, Ji Young Park, Jieun Oh
POEMS syndrome is a rare paraneoplastic syndrome and there are few reports of polyneuropathy and monoclonal gammopathy associated with kidney dysfunction. Here, we report a case of POEMS syndrome with recurrent acute kidney injury (AKI). A 52-year-old man presented with bilateral aggravating paresthesia and latermotor weakness of the lower extremities accompanied by repeated elevation of serum creatinine. The patient was finally diagnosed with POEMS syndrome on the basis of fulfilling the two mandatory major criteria (polyneuropathy and monoclonal gammopathy), one other major criterion (sclerotic bone lesion), and several minor criteria...
June 2016: Electrolyte & Blood Pressure: E & BP
Jorge Vega
POEMS syndrome is characterized by Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal protein and Skin changes. We report a woman with the syndrome, who had peripheral polyneuropathy, osteosclerotic myeloma, monoclonal IgA elevation, hypothyroidism, hypogonadotrophic hypogonadism, hyperprolactinemia, adrenal insufficiency, hepatosplenomegaly, lymphadenopathy, thyroid and parotid enlargement, Castleman’s disease, papilledema, stiff and hyperpigmented skin, white nails, clubbing, ascites and chronic diarrhea...
April 2016: Revista Médica de Chile
C P Fan, J X Liang, J M Hou
No abstract text is available yet for this article.
July 1, 2016: Zhonghua Nei Ke za Zhi [Chinese Journal of Internal Medicine]
Kyari Sumayin Ngamdu, Alireza Torabi, Nabeel Badri, Mohammed Teleb, Sumit Gaur
Multiple myeloma is a clonal hematopoietic neoplasm characterized by the proliferation of malignant plasma cells and associated end-organ damage, most notably lytic lesions in the bones. Osteosclerotic myeloma is an unusual variant of the disease in which the skeletal involvement is characterized by sclerotic lesions instead of classical lytic lesions. The disease can be associated with paraneoplastic symptoms, which have been given the acronym POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M protein, skin changes)...
July 2016: Proceedings of the Baylor University Medical Center
A Birkenbach, F Kühlhorn, M Grube, H Helbig, M A Gamulescu
The POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, skin changes) is a rare paraneoplastic syndrome based on a clonal plasma cell disorder. Optic disc edema (ODE) is a frequent ocular sign in POEMS syndrome. The cause of the ODE has not yet been entirely clarified. This article reports the case of a 62-year-old male suffering from POEMS syndrome with a bilateral ODE.
June 30, 2016: Der Ophthalmologe: Zeitschrift der Deutschen Ophthalmologischen Gesellschaft
C Wang, X-F Huang, Q-Q Cai, X-X Cao, M-H Duan, H Cai, D-B Zhou, J Li
POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes) is a multisystem disorder with a good long-term prognosis. In its dozens of clinical features, those with independent prognostic value are still not well characterized. We retrospectively included 362 patients with newly diagnosed POEMS syndrome at our institute from 2000 to 2015. On the basis of a randomized sample splitting, we first identified four baseline clinical variables, including age >50 years (hazards ratio (HR) 4...
June 24, 2016: Leukemia: Official Journal of the Leukemia Society of America, Leukemia Research Fund, U.K
Hongbo Yang, Xufei Huang, Qianqian Cai, Chen Wang, Xinxin Cao, Daobin Zhou, Jian Li
BACKGROUND: POEMS syndrome is a rare paraneoplastic syndrome due to an underlying plasma cell neoplasm. Hypogonadism is the most common endocrine abnormality in POEMS syndrome. There is no data about improvement of hypogonadism and sexual dysfunction after appropriate treatment of POEMS syndrome so far. In this single-center prospective study, the efficacy of low-dose lenalidomide and dexamethasone combination therapy in the improvement of sexual and gonadal function in POEMS syndrome was evaluated...
2016: Orphanet Journal of Rare Diseases
Xin-Xin Cao, Qi Meng, Yue-Ying Mao, Wei Su, Jun-Feng Zhen, Kai-Ni Shen, Chun-Lan Zhang, Xu-Fei Huang, Ming-Hui Duan, Wei Zhang, Tie-Nan Zhu, Hua-Cong Cai, Miao Chen, Dao-Bin Zhou, Jian Li
OBJECTIVES: We retrospectively evaluated the clinical features, serum levels of IgM, and prevalence of IgM related diseases in patients with serum immunofixation electrophoresis (sIFE) confirmed IgM monoclonal gammopathy at our center. METHODS: We included patients with sIFE confirmed IgM monoclonal gammopathy between January 2008 and December 2014 in this retrospective study. We evaluated clinical data, sIFE, serum IgM levels, and diagnosis. RESULTS: In total, 7107 patients had sIFE confirmed monoclonal gammopathy, with 377 (5...
July 2016: Leukemia Research
I Hernández Aragüés, A Pulido Pérez, C Ciudad Blanco, V Parra Blanco, R Suárez Fernández
POEMS syndrome is a paraneoplastic manifestation associated with hematopoietic disorders such as multiple myeloma and Castleman disease. POEMS is an acronym for the main clinical features of the syndrome, namely, Polyneuropathy, Organomegaly, Endocrinopathy, M protein, and Skin abnormalities. Glomeruloid hemangiomas are considered to be a specific clinical marker of POEMS syndrome. However, while they are not pathognomonic, their presence should raise suspicion of this syndrome or alert clinicians to its possible future development, as these lesions can appear years before the onset of the syndrome...
May 18, 2016: Actas Dermo-sifiliográficas
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