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https://www.readbyqxmd.com/read/29907605/transthyretin-amyloid-polyneuropathies-mimicking-a-demyelinating-polyneuropathy
#1
Pierre Lozeron, Louise-Laure Mariani, Pauline Dodet, Guillemette Beaudonnet, Marie Théaudin, Clovis Adam, Bertrand Arnulf, David Adams
OBJECTIVE: To clearly define transthyretin familial amyloid polyneuropathies (TTR-FAPs) fulfilling definite clinical and electrophysiologic European Federation of Neurological Societies/Peripheral Nerve Society criteria for chronic inflammatory demyelinating polyneuropathy (CIDP). METHODS: From a cohort of 194 patients with FAP, 13 of 84 patients (15%) of French ancestry had late-onset demyelinating TTR-FAP. We compared clinical presentation and electrophysiology to a cohort with CIDP and POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes) syndrome...
June 15, 2018: Neurology
https://www.readbyqxmd.com/read/29877235/-successful-treatment-of-poems-syndrome-associated-pulmonary-hypertension-with-lenalidomide-and-dexamethasone-therapy
#2
Daisuke Nakamura, Maiko Hayashida, Kayoko Kubota, Eiji Matsuura, Tomohisa Tabuchi, Naosuke Arima, Hirosaka Inoue, Makoto Yoshimitsu, Kenji Ishitsuka
POEMS syndrome is often complicated by pulmonary hypertension. The standard therapy for patients with POEMS syndrome is high-dose chemotherapy followed by autologous stem cell transplantation. However, the safety of high-dose chemotherapy for patients complicated with pulmonary hypertension remains unclear, and the optimal therapy for these patients is yet to be establishment. Herein, we report the case of a 54-year-old woman with POEMS syndrome accompanied by pulmonary hypertension. We successfully and safely performed lenalidomide and dexamethasone (Ld) therapy followed by high-dose chemotherapy and autologous stem cell transplantation, which improved her pulmonary hypertension...
2018: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/29861185/cutaneous-mycobacterium-chelonae-infection-following-autologous-peripheral-blood-stem-cell-transplantation-for-poems-syndrome
#3
Yoshinori Hashimoto, Ayano Ikeda, Yusuke Tokuyasu, Hiromi Omura, Takayuki Tanaka
Although hematopoietic stem cell transplantation (HSCT) may increase the curability of refractory hematologic diseases, it requires complication management due to a long-term immunocompromised state. We experienced a case who received an autologous peripheral blood stem cell transplantation (Auto-PBSCT) for POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) and developed cutaneous Mycobacterium chelonae infection. It is clear that attention needs to be paid to prevent bacterial, fungal and viral infection after HSCT...
May 31, 2018: Journal of Infection and Chemotherapy: Official Journal of the Japan Society of Chemotherapy
https://www.readbyqxmd.com/read/29797921/-contribution-of-jiusheng-by-zhou-meisheng-to-moxibustion
#4
Zijian Wu, Huangan Wu, Ling Hu, Qing Yu, Jie Wang
Mr. ZHOU Meisheng was a famous acupuncturist in China. He succeeded the acupuncture from his family in childhood, and practiced acupuncture strictly with insightful and original perspective. The Jiusheng , one of his magnum opus, proposed innovative ideas in the theory and clinical practice of moxibustion, such as revival of moxibustion, three phases of moxibustion sensation, moxibustion for heat syndromes. In this article, the contribution of Jiusheng to moxibustion was discussed from background of compiling this book, poem and medical cases, explanation on moxibustion theory and practice and its effects on later ages...
May 12, 2018: Zhongguo Zhen Jiu, Chinese Acupuncture & Moxibustion
https://www.readbyqxmd.com/read/29739345/atypical-central-retinal-artery-occlusion-as-the-first-presentation-of-poems-syndrome-a-case-report
#5
Panitha Jindahra, Charungthai Dejthevaporn, Pimjai Niparuck, Jariya Waisayarat, Piyaphon Cheecharoen, Thanatporn Threetong, Purit Petpiroon, Tharikarn Sujirakul, Anuchit Poonyathalang, Kavin Vanikieti
BACKGROUND: POEMS syndrome is a plasma cell disorder, which clinically manifests from paraneoplastic syndrome: polyneuropathy, organomegaly, endocrinopathy, monoclonal plasma cell disorder, and skin changes. The most common ocular manifestation is optic disc swelling, whereas other ocular manifestations; cystoid macular edema, serous macular detachment, venous sinus thrombosis, infiltrative orbitopathy, uveitis, neovascularization of the disc, peripapillary choroidal neovascularization and optic disc drusen, had also been reported...
May 8, 2018: BMC Neurology
https://www.readbyqxmd.com/read/29732477/effectiveness-of-rituximab-containing-treatment-regimens-in-idiopathic-multicentric-castleman-disease
#6
Yujun Dong, Lu Zhang, Lin Nong, Lihong Wang, Zeyin Liang, Daobin Zhou, David C Fajgenbaum, Hanyun Ren, Jian Li
Human herpes virus type 8 (HHV-8)-negative, idiopathic multicentric Castleman disease (iMCD) is a rare lymphoproliferative disease often involving constitutional symptoms, cytopenias, and multiple organ system dysfunction. In China, the majority of MCD cases are HHV-8 negative. Given that siltuximab, the only FDA-approved treatment for iMCD is not available in China; rituximab- and cyclophosphamide-containing regimens are often used in the treatment of Chinese iMCD patients. To evaluate the efficacy of rituximab in this rare and heterogeneous disease, clinical and pathological data from 27 cases of iMCD were retrospectively analyzed from two large medical centers in China...
May 7, 2018: Annals of Hematology
https://www.readbyqxmd.com/read/29702119/glomeruloid-hemangioma-associated-with-tafro-syndrome
#7
Aya Shinozaki-Ushiku, Takaaki Higashihara, Masako Ikemura, Junichiro Sato, Masaomi Nangaku, Tetsuo Ushiku, Masashi Fukayama
Glomeruloid hemangioma is a rare cutaneous lesion which has been considered as a specific cutaneous marker of POEMS syndrome. Herein, we present the first case of glomeruloid hemangioma associated with TAFRO syndrome, a unique variant of idiopathic multicentric Castleman's disease (iMCD). The patient is a 74-year-old female presented with fever, cervical lymphadenopathy, thrombocytopenia, bilateral pleural effusions and ascites. Biopsy of the lymph node revealed MCD-like histology and bone marrow biopsy showed mild reticulin fibrosis, consistent with TAFRO syndrome...
April 24, 2018: Human Pathology
https://www.readbyqxmd.com/read/29691905/hepatobiliary-and-pancreatic-obliterative-portal-venopathy-found-in-castleman-disease-variant-of-poems-syndrome
#8
L Zhang, H Gao, Y Feng, G Wang, X Zhang, Z Li
No abstract text is available yet for this article.
April 24, 2018: Journal of Gastroenterology and Hepatology
https://www.readbyqxmd.com/read/29687294/endoscopic-therapies-for-gastroparesis
#9
REVIEW
Andrew Su, Jeffrey L Conklin, Alireza Sedarat
PURPOSE OF REVIEW: Gastroparesis remains a difficult-to-treat disease with limited therapeutic options. Though patients often have a common syndrome of stereotypic symptoms, the underlying pathophysiology is heterogeneous, often leading to variable treatment responses. Due to limitations in medical and surgical therapies, endoscopic options have been increasingly explored. These options can be broadly categorized into pyloric-directed therapy, non-pyloric-directed therapy, and nutritional support...
April 23, 2018: Current Gastroenterology Reports
https://www.readbyqxmd.com/read/29665938/-research-and-applications-progress-of-lenalidomide-in-relapsed-refractory-blood-system-diseases-review
#10
Wen-Jing Fan, Zhi-Qiao Fan, Tao Wu, Hai Bai
Lenalidomide, a novel immunomodulatory agent, is a kind of thalidomide derivatives, which shows a good efficacy and safety for hematological system diseases. This review is aimed to evaluate the efficacy and safety of lenalidomide in treatment of patients with multiple myeloma, chronic lymphocytic leukemia, acute myeloid leukemia, non-Hodgkin's lymphoma, classical Hodgkin's lymphoma and POEMS syndrome at their replased or refractory state. At the same time, this review focuses on the newest clinical research and the latest application progress of lenalidomide for relapsed or refractory hematological system diseases...
April 2018: Zhongguo Shi Yan Xue Ye Xue za Zhi
https://www.readbyqxmd.com/read/29650525/flushing-disorders-associated-with-gastrointestinal-symptoms-part-1-neuroendocrine-tumors-mast-cell-disorders-and-hyperbasophila
#11
REVIEW
Vaibhav Rastogi, Devina Singh, Joseph J Mazza, Dennis Yang, Dipendra Parajuli, Steven H Yale
Flushing is the subjective sensation of warmth accompanied by visible cutaneous erythema occurring throughout the body with a predilection for the face, neck, pinnae, and upper trunk where the skin is thinnest and cutaneous vessels are superficially located and in greatest numbers. Flushing can be present in either a wet or dry form depending upon whether neural-mediated mechanisms are involved. Activation of the sympathetic nervous system results in wet flushing, accompanied by diaphoresis, due to concomitant stimulation of eccrine sweat glands...
April 12, 2018: Clinical Medicine & Research
https://www.readbyqxmd.com/read/29622867/autologous-stem-cell-transplantation-for-multiple-myeloma-single-centre-experience-from-north-india
#12
Pankaj Malhotra, Uday Yanamandra, Alka Khadwal, Gaurav Prakash, Deepesh Lad, Arjun D Law, Harshit Khurana, M U S Sachdeva, Praveen Bose, Reena Das, Neelam Varma, Subhash Varma
Autologous stem cell transplantation (ASCT) is considered as standard of care in patients with multiple myeloma (MM) patients aged 65 years or younger. We analyzed data of 94 patients of plasma cell dyscrasias who underwent 95 autologous transplants at our institute from October 2003 to Aug 2016. Other than 76 patients of newly diagnosed multiple myeloma, we also transplanted two patients of POEMS syndrome, two patients of plasma cell leukemia, three patients of concurrent light chain deposition disease, three patients of multifocal plasmacytomas, and eight patients of isolated light chain myeloma...
April 2018: Indian Journal of Hematology & Blood Transfusion
https://www.readbyqxmd.com/read/29603764/a-prospective-phase-ii-study-of-low-dose-lenalidomide-plus-dexamethasone-in-patients-with-newly-diagnosed-polyneuropathy-organomegaly-endocrinopathy-monoclonal-gammopathy-and-skin-changes-poems-syndrome
#13
Jian Li, Xu-Fei Huang, Qian-Qian Cai, Chen Wang, Hao Cai, Hao Zhao, Lu Zhang, Xin-Xin Cao, Robert Peter Gale, Dao-Bin Zhou
Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome is a rare plasma dyscrasia without standard treatment. This phase II prospective trial evaluates the safety and response of 12 cycles of low dose lenalidomide (10 mg) plus dexamethasone (Rdex) in patients with newly diagnosed POEMS syndrome. Forty-one patients (28 men) were enrolled and the median age at diagnosis was 49 years (range, 21-70 years). Twenty-one patients (46%) achieved complete hematologic response and the neurologic response rate was 95%...
March 31, 2018: American Journal of Hematology
https://www.readbyqxmd.com/read/29599110/long-term-evaluation-of-physical-improvement-and-survival-of-autologous-stem-cell-transplantation-in-poems-syndrome
#14
LETTER
Chikako Ohwada, Emiko Sakaida, Chika Kawajiri-Manako, Yuhei Nagao, Nagisa Oshima-Hasegawa, Emi Togasaki, Tomoya Muto, Shokichi Tsukamoto, Shio Mitsukawa, Yusuke Takeda, Naoya Mimura, Masahiro Takeuchi, Naomi Shimizu, Sonoko Misawa, Tohru Iseki, Satoshi Kuwabara, Chiaki Nakaseko
No abstract text is available yet for this article.
May 10, 2018: Blood
https://www.readbyqxmd.com/read/29547451/retinal-microstructure-in-poems-syndrome
#15
Ryutaro Tomita, Tetsuju Sekiryu, Hiroaki Shintake, Kuniharu Saito
PURPOSE: To report a 67-year-old woman with polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome, showing bilateral serous retinal detachment. The retinal lesion was evaluated by multimodal imaging using spectral domain optical coherence tomography and an adaptive optics (AO) camera. METHODS: A case report. RESULTS: Optical coherence tomography showed hyperreflective foci mainly in the inner retina in both eyes...
March 15, 2018: Retinal Cases & Brief Reports
https://www.readbyqxmd.com/read/29515067/-sporadic-late-onset-nemaline-myopathy-with-monoclonal-gammopathy-of-undetermined-significance-mimicking-poems-syndrome
#16
Takashi Kanamori, Shigeru Kusumoto, Kenji Okita, Shinya Hagiwara, Chie Kato, Takahiro Nakashima, Satsuki Murakami, Tomoko Narita, Asahi Ito, Masaki Ri, Takashi Ishida, Hirokazu Komatsu, Noriyuki Matsukawa, Shinsuke Iida
A 40-year-old female presented with a skin rash, hepatosplenomegaly, hypothyroidism, IgG-λ monoclonal gammopathy, slightly elevated serum VEGF levels, and >1-year history of weakness in the posterior cervical muscles. Based on these symptoms and her clinical course, she was suspected of having POEMS syndrome. However, because there was no sign of peripheral neuropathy (PN), the criteria for the diagnosis of POEMS syndrome were not met. Consequently, she continued follow-up and was under close observation as an outpatient...
2018: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/29511110/poems-neuropathy-optimising-diagnosis-and-management
#17
REVIEW
Stephen Keddie, Shirley D'Sa, David Foldes, Aisling S Carr, Mary M Reilly, Michael P T Lunn
POEMS syndrome is a rare and disabling autoinflammatory condition characterised by a typical peripheral neuropathy and the presence of a monoclonal plasma cell disorder. The acronym 'POEMS' represents the complex and multisystem features of the disease, including polyneuropathy, organomegaly, endocrinopathy, a monoclonal plasma cell disorder and skin disease. The diagnosis of POEMS is a significant challenge because of the heterogeneity of clinical presentations and variation of POEMS features. Patients are often misdiagnosed with another cause of inflammatory neuropathy and receive one or more ineffective immunomodulatory medications, resulting in delayed diagnosis and further clinical deterioration before a diagnosis is made...
March 6, 2018: Practical Neurology
https://www.readbyqxmd.com/read/29489695/a-case-report-of-poems-syndrome-with-renal-involvement-as-immunotactoid-glomerulopathy
#18
Yan Wang, Yu Yan, Zhun Sui, Bao Dong, Li Zuo
RATIONALE: POEMS syndrome is a rare multi-system disorder, which sometimes involves the kidney. Immunotactoid glomerulopathy (ITG) is an uncommon glomerular disease resulted from deposits of immunoglobulins and its derivatives. ITG caused by POEMS syndrome is rarely reported. PATIENT CONCERNS: A 63-year-old man was presented with acute kidney injury. In addition, it's found that he had abnormal serum free κ /λ ratio, polyneuropathy, Castleman's disease, organomegaly, endocrinopathy and skin changes...
March 2018: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29468524/tafro-syndrome-with-refractory-thrombocytopenia-responding-to-tocilizumab-and-romiplostim-a-case-report
#19
Shoko Noda-Narita, Keiichi Sumida, Akinari Sekine, Junichi Hoshino, Koki Mise, Tatsuya Suwabe, Noriko Hayami, Masayuki Yamanouchi, Toshiharu Ueno, Hiroki Mizuno, Masahiro Kawada, Rikako Hiramatsu, Eiko Hasegawa, Naoki Sawa, Kenmei Takaichi, Kenichi Ohashi, Takeshi Fujii, Yoshifumi Ubara
Thrombocytopenia, anasarca, fever, reticulin fibrosis, organomegaly (TAFRO) syndrome is a unique clinicopathologic subtype of multicentric Castleman's disease that has recently been identified in Japan. However, little is known about its renal histological changes and the optimal treatment for TAFRO syndrome. An 80-year-old Japanese woman was admitted to our hospital for evaluation of severe anasarca and weight gain (10 kg in a month). She had polyneuropathy, monoclonal plasma cell proliferative disorder with positive kappa M-protein, a sclerotic bone lesion, elevation of vascular endothelial growth factor (VEGF), skin changes, and extravascular volume overload, which fulfilled the diagnostic criteria for POEMS (polyneuropathy, organomegaly, endocrinopathy, and monoclonal protein, skin changes) syndrome...
May 2018: CEN Case Reports
https://www.readbyqxmd.com/read/29467805/reduction-of-optic-disc-oedema-by-bortezomib-and-dexamethasone-followed-by-autologous-peripheral-blood-stem-cell-transplantation-in-patient-with-poems-syndrome
#20
Takehito Iwase, Hirotaka Yokouchi, Sonoko Misawa, Toshiyuki Oshitari, Takayuki Baba, Satoshi Kuwabara, Shuichi Yamamoto
The authors present findings in a 39-year-old man with polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome with bilateral optic disc oedema. He was successfully treated with bortezomib and dexamethasone followed by autologous peripheral blood stem cell transplantation. The peripapillary retinal thickness was reduced in the optical coherence tomographic (OCT) images along with a decrease of the serum vascular endothelial growth factor (VEGF) levels. The authors recommend OCT to monitor the changes in the signs of POEMS syndrome after treatments...
February 2018: Neuro-ophthalmology
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