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https://www.readbyqxmd.com/read/29665938/-research-and-applications-progress-of-lenalidomide-in-relapsed-refractory-blood-system-diseases-review
#1
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
#2
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
#3
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-poems-syndrome
#4
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
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 (10mg) 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%. The median serum VEGF declined from 5155 pg/ml (range, 534-14328 pg/ml) to 832 pg/ml (95-6254 pg/ml) after therapy...
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
#5
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.
March 29, 2018: Blood
https://www.readbyqxmd.com/read/29547451/retinal-microstructure-in-poems-syndrome
#6
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
#7
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
#8
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
#9
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
#10
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
#11
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
https://www.readbyqxmd.com/read/29430125/significance-of-18f-sodium-fluoride-positron-emission-tomography-in-characterization-of-poems-osteosclerotic-lesions-better-than-18f-fluorodeoxyglucose-positron-emission-tomography
#12
Fahad Abdul Rehman Marafi, Rashid Rasheed, Sharjeel Usmani, Fareeda Alkandari, Anam Iqbal
Crow-Fukase syndrome (POEMS syndrome) is a rare systemic paraneoplastic syndrome. Bone lesions are manifested by sclerotic osteoblastic lesions often associated with bone pain. Characterization of osseous lesions is always crucial for clinical correlation and better patient management. We present a case where 18F-fluorodeoxyglucose positron emission tomography (18F-FDG PET/CT) was unable to characterize a clinically symptomatic patient, and 18F-fluoride PET/CT showed excellent characterization of osteosclerotic lesions...
January 2018: Indian Journal of Nuclear Medicine: IJNM: the Official Journal of the Society of Nuclear Medicine, India
https://www.readbyqxmd.com/read/29409882/the-efficacy-and-long-term-outcomes-of-autologous-stem-cell-transplantation-in-poems-syndrome-a-nation-wide-survey-in-japan
#13
Chika Kawajiri-Manako, Emiko Sakaida, Chikako Ohwada, Toshihiro Miyamoto, Taichi Azuma, Jun Taguchi, Takehiko Mori, Yuichi Hasegawa, Tadakazu Kondo, Toshiaki Yujiri, Makoto Yoshimitsu, Kazunori Imada, Shingo Kurahashi, Kaoru Kahata, Tatsuo Ichinohe, Makoto Hirokawa, Yoshiko Atsuta, Chiaki Nakaseko
POEMS syndrome is a rare plasma cell dyscrasia presenting with polyneuropathy, λ-type M protein, VEGF elevation, and systemic manifestations. The standard treatment has not been established, but autologous stem cell transplantation (ASCT) has exhibited effectiveness in this syndrome. However, the efficacy and long-term outcomes of ASCT have not been systematically studied. To clarify the efficacy and long-term outcomes of ASCT-treated patients in Japan, we performed a multicenter retrospective study assessing the clinical course of patients registered to the Japan Society for Hematopoietic Cell Transplantation TRUMP database...
January 31, 2018: Biology of Blood and Marrow Transplantation
https://www.readbyqxmd.com/read/29397648/gastric-peroral-endoscopic-myotomy
#14
REVIEW
Hyunsoo Chung, Mouen A Khashab
Gastroparesis (GP) is a syndrome characterized by delayed gastric emptying in the absence of mechanical obstruction of the stomach or proximal small bowel. Currently available dietary and medical therapies are limited and have suboptimal efficacy. Pylorus-directed therapies have showed promising results. Gastric peroral endoscopic myotomy (G-POEM) has been reported for the treatment of GP refractory to standard therapy with promising results. This article reviews the current applications and results of G-POEM for the treatment of refractory GP...
January 2018: Clinical Endoscopy
https://www.readbyqxmd.com/read/29395638/lenalidomide-induced-ischemic-cerebrovascular-disease-in-polyneuropathy-organomegaly-endocrinopathy-monoclonal-gammopathy-and-skin-changes-syndrome
#15
Akihiko Mitsutake, Hideyuki Matsumoto, Keiko Hatano, Koreaki Irie, Nobuhiro Tsukada, Hideji Hashida
We describe the case of a 34-year-old woman with polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome. She developed transient ischemic attack after the introduction of lenalidomide plus dexamethasone (Rd) therapy despite no vascular risk factors. Magnetic resonance and computed tomography angiographies showed bilateral internal carotid artery stenosis. Rd therapy was suspended because of its thromboembolic risk. She had been neurologically stable during the suspension of Rd therapy...
January 29, 2018: Journal of Stroke and Cerebrovascular Diseases: the Official Journal of National Stroke Association
https://www.readbyqxmd.com/read/29383495/per-oral-endoscopic-myotomy-for-esophageal-achalasia-in-a-case-of-allgrove-syndrome
#16
Jun Nakamura, Takuto Hikichi, Haruhiro Inoue, Ko Watanabe, Hitomi Kikuchi, Tadayuki Takagi, Rei Suzuki, Mitsuru Sugimoto, Naoki Konno, Yuichi Waragai, Hiroyuki Asama, Mika Takasumi, Yuki Sato, Hiroki Irie, Katsutoshi Obara, Hiromasa Ohira
Allgrove syndrome, also known as Triple A syndrome, is a rare autosomal recessive genetic disease characterized by three signs: esophageal achalasia, adrenocorticotropic hormone refractoriness, and alacrima. A 31-year-old male presented to our hospital for treatment of difficulty swallowing caused by esophageal achalasia. Because he had complicating alacrima, a neurologic disease, and a family history of consanguineous marriage, a genetic neurologic disease was suspected. Then, a mutation in the achalasia-addisonianism-alacrima syndrome gene was identified...
January 30, 2018: Clinical Journal of Gastroenterology
https://www.readbyqxmd.com/read/29346810/-poems-syndrome
#17
Ricarda Schwarz, Marius Horger
No abstract text is available yet for this article.
February 2018: RöFo: Fortschritte Auf Dem Gebiete der Röntgenstrahlen und der Nuklearmedizin
https://www.readbyqxmd.com/read/29333225/a-rare-cause-of-pericardial-effusion-and-ascites-poems-syndrome
#18
Bilal Katipoglu, Zeynep Katipoğlu, Ihsan Ates, Gokhan Yirgin, Burak Furkan Demir, Fatih Acehan
No abstract text is available yet for this article.
December 22, 2017: Hematology Reports
https://www.readbyqxmd.com/read/29319491/cerebral-pseudotumor-as-the-first-manifestation-of-poems-syndrome-a-case-report
#19
Audrey Benyamine, Clemence Delteil, Nicolas Macagno, Pauline Belenotti, Bertrand Dussol, Nadine Girard, Pierre-Jean Weiller, Brigitte Granel, Laurent Daniel
No abstract text is available yet for this article.
March 2018: Clinical Neuropathology
https://www.readbyqxmd.com/read/29288001/-a-poems-syndrome-revealed-by-a-blue-toe-syndrome
#20
P Decker, J Galland, J Risse, M Claudin, S Mohamed, A Perrot, D Wahl
INTRODUCTION: Arterial and venous thromboses occur in almost one in five patients with POEMS syndrome and usually in macrocirculation. CASE REPORT: We report a 67-year-old male with a POEMS syndrome who presented initially with a blue toe syndrome. He complained of Raynaud's syndrome and left foot paresthesia. Physical examination showed gynecomastia, lymphadenopathies and skin lesions. Cardiovascular investigations excluded atrial fibrillation, unstable atherosclerotic lesions and vascular calcifications...
February 2018: La Revue de Médecine Interne
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