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Poems syndrome relapse

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
Michelle L Mauermann
PURPOSE OF REVIEW: This article reviews the spectrum of neurologic complications associated with lymphoma, leukemia, and paraproteinemic disorders. While leptomeningeal metastasis is the most common complication of lymphoma and leukemia and peripheral neuropathy is the most common complication of paraproteinemic disorders, clinicians need to be familiar with the diverse neurologic complications of these disorders. RECENT FINDINGS: Lymphomatous nervous system involvement can be difficult to diagnose, especially when it is the presenting symptom...
June 2017: Continuum: Lifelong Learning in Neurology
Rahma Warsame, Uday Yanamandra, Prashant Kapoor
POEMS syndrome is a paraneoplastic disorder secondary to an underlying plasma cell dyscrasia. By definition, all patients with POEMS syndrome must display polyneuropathy and monoclonal plasma cell disorder. In addition, at least one major criterion (Castleman's disease, sclerotic bone lesions, or vascular endothelial growth factor elevation) and one minor criterion (organomegaly, extravascular volume overload, endocrinopathy, skin changes, papilledema, thrombocytosis, or polycythemia) are required for diagnosis...
April 2017: Current Hematologic Malignancy Reports
A Marchal, F Charlotte, P Maksud, J Haroche, F Lifferman, M Miyara, S Choquet, Z Amoura, F Cohen Aubart
INTRODUCTION: Sarcoidosis is a systemic granulomatous disorder of unknown cause. Apparition or flare of previously diagnosed sarcoidosis following hematopoietic stem cell transplantation (HSCT) has rarely been reported. OBSERVATION: We report a 62-year-old woman who presented a radiological flare of sarcoidosis post-autologous stem cell transplantation for a POEMS syndrome. Imaging findings and lymph node histology, which revealed non-caseating granuloma, were consistent with the sarcoidosis diagnosis...
September 2017: La Revue de Médecine Interne
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, Laurant 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. One hundred and twenty-seven patients underwent an autologous stem cell transplantation between 1997-2010 with a median age of 50 years (range 26-69 years). Median time from diagnosis to autologous stem cell transplantation was 7...
January 2017: Haematologica
S Misawa, Y Sato, K Katayama, H Hanaoka, S Sawai, M Beppu, F Nomura, K Shibuya, Y Sekiguchi, Y Iwai, K Watanabe, H Amino, C Ohwada, M Takeuchi, E Sakaida, C Nakaseko, S Kuwabara
OBJECTIVE: POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes) syndrome is a rare multisystem disease characterised by plasma cell dyscrasia and overproduction of vascular endothelial growth factor (VEGF). VEGF is assumed to be useful in monitoring disease activity, because VEGF levels usually decrease after treatment. However, there is no study to investigate whether the extent of decrease in VEGF correlates with clinical outcome. We tested the predictive efficacy of serum VEGF levels in POEMS syndrome...
2015: BMJ Open
Chen Wang, Wei Su, Qian-Qian Cai, Hao Cai, Wei Ji, Qian Di, Ming-Hui Duan, Xin-Xin Cao, Dao-Bin Zhou, Jian Li
POEMS syndrome is a rare plasma cell dyscrasia. Serum concentrations of the monoclonal protein in this disorder are typically low, and inapplicable to monitor disease activity in most cases, resulting in limited practical and prognostic values. Novel immunoassays measuring isotype-specific heavy/light chain (HLC) pairs showed its utility in disease monitoring and outcome prediction in several plasma cell dyscrasias. We report results of HLC measurements in 90 patients with POEMS syndrome. Sixty-six patients (73%; 95% confidence interval, 63-82%) had an abnormal HLC ratio at baseline...
July 2016: European Journal of Haematology
Il-Young Jang, Dok Hyun Yoon, Shin Kim, Kyoungmin Lee, Kwang-Kuk Kim, Young-Min Lim, Won-Ki Min, Cheolwon Suh
BACKGROUND: POEMS syndrome is a rare paraneoplastic syndrome associated with plasma cell dyscrasia. High-dose chemotherapy followed by autologous stem cell transplantation has shown encouraging efficacy in the treatment of patients with POEMS syndrome. However, there are minimal reports on clinical outcomes after autologous stem cell transplantation for patients with advanced disease and very poor performance status. METHODS: We retrospectively evaluated 9 advanced POEMS syndrome patients, who had an Eastern Cooperative Oncology Group performance status score of 3 or 4, and were treated with high-dose melphalan therapy followed by autologous stem cell transplantation from 2004 to 2011...
March 2014: Blood Research
D Adams
Improvement of therapeutic strategies for peripheral neuropathies requires multicentric clinical trials. For chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), a randomized controlled multicentric study compared IgIV to pulses of methylprednisolone (MP) given for 6 months. The primary endpoint was treatment discontinuation due to inefficacy or intolerance; 45 patients were enrolled: more patients had interrupted MP than IVIg, usually because of inefficacy. A multicentric randomized clinical trial (PREDICT) evaluated long-term remission of CIDP after short-term corticosteroid therapy (pulses of dexamethasone or prednisolone); 39 patients were enrolled: 26% achieved cure or remission, a relapse occurred in 50% after a delay of 11 to 17 months...
December 2013: Revue Neurologique
Kota Ohashi, Akihiro Yokoyama, Toshiya Kagoo, Toru Hosoda, Ryusuke Yamamoto, Mie Yoneda, Masahisa Kudo, Saigen Boku, Hironori Ueno, Takahiro Yano
We report a 53-year-old male with POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) syndrome who relapsed after autologous peripheral stem cell transplantation (ASCT), but responded extremely well to lenalidomide (LEN) plus low dose dexamethasone (Ld) therapy. The patient had been diagnosed with POEMS syndrome in November 2006, and underwent ASCT in February 2007. In July 2011, he developed respiratory distress, generalized edema, and massive bilateral pleural effusion...
July 2013: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
Satoshi Kuwabara
POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein, and skin changes) syndrome is a rare multiorgan disorder associated with plasma cell dyscrasia and overproduction of vascular endothelial growth factor (VEGF). VEGF presumably plays an important role in the pathogenesis of the syndrome by its strong action on neo-vasculization and increased vascular permeability. POEMS syndrome is potentially fatal disease, and patients' quality of life deteriorates because of progressive neuropathy and/or massive peripheral edema or pleural effusion/ascites...
May 2013: Nihon Rinsho. Japanese Journal of Clinical Medicine
Bruno Royer, Lavinia Merlusca, Julie Abraham, Lucile Musset, Julien Haroche, Sylvain Choquet, Xavier Leleu, Catherine Sebban, Olivier Decaux, Lionel Galicier, Muriel Roussel, Christian Recher, Anne Banos, Isabelle Guichard, Jean-Marie Brisseau, Pascal Godmer, Olivier Hermine, Gaël Deplanque, Thierry Facon, Bouchra Asli, Véronique Leblond, Jean-Paul Fermand, Jean Pierre Marolleau, Arnaud Jaccard
POEMS syndrome is a rare disorder characterized by polyneuropathy, monoclonal gammopathy, multiorgan involvement, and elevated vascular endothelial growth factor levels. Localized bone lesions require irradiation, whereas young patients with disseminated disease receive intensive treatment with stem cell support. Treatment of older and non responding patients is not yet standardized. We report the use of a combination of lenalidomide and dexamethasone in 20 patients with POEMS syndrome. Four patients were newly diagnosed, and 16 had relapsed or progressed after treatment...
March 2013: American Journal of Hematology
Norina Tanaka, Kentaro Yoshinaga, Chihiro Asano, Hiroshi Kazama, Takamitsu Okamura, Masayuki Shiseki, Naoki Mori, Masanao Teramura, Kiyoko Kusakabe, Shuji Sakai, Toshiko Motoji
Many patients with POEMS syndrome have osteosclerotic plasmacytoma. Radiation therapy is useful for patients who have localized lesions, although chemotherapy is necessary for patients who have widespread lesions. Thus, evaluation of these lesions is important to determine the therapeutic strategy. We evaluated the activities of lesions in two patients with POEMS syndrome by (18)F-FDG positron emission tomography (PET)/computed tomography (CT) scan. In the first patient, PET/CT scan revealed osteosclerotic lesions, which were not detected by Ga-scintigraphy or plain X-ray...
December 2012: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
Z Adam, L Pour, M Krejčí, L Zahradová, P Szturz, R Koukalová, Z Rehák, T Nebeský, R Hájek, Z Král, J Mayer
UNLABELLED: Lenalidomide has been licenced for the treatment of multiple myeloma and, in 2012, it is used as a standard treatment of relapses of the disease. Literature contains a number of publications on the effects of lenalidomide in myelodysplastic syndrome, in malignant lymphomas and chronic B lymphocytic leukaemia. The effects of the drug in rare diseases, however, have not been investigated so far. In this paper, we summarize our experience with lenalidomide in rare blood disorders...
November 2012: Vnitr̆ní Lékar̆ství
Rahma Warsame, Ingrid E Kohut, Angela Dispenzieri
POEMS syndrome is a rare paraneoplastic condition related to myeloma. Because it is rare, there is very little known about treatment options. The use of potentially neurotoxic chemotherapeutic drugs is of concern in a disease whose major manifestation is neuropathy. Herein, we describe an extraordinary response to the combination of cyclophosphamide, bortezomib, and dexamethasone (CyBorD) in a patient with relapsed, life-threatening POEMS syndrome.
June 2012: European Journal of Haematology
Antonella Stefanelli, Giorgio Treglia, Lucia Leccisotti, Luca Laurenti, Marco Luigetti, Mario Sabatelli, Alessandro Giordano
We report a case of a patient with relapse of POEMS syndrome (peripheral neuropathy, organomegaly, endocrinopathy, monoclonal plasma proliferative disorder, skin changes) that occurred 6 years after an autologous peripheral blood stem cell transplantation. F-18 FDG PET/CT showed several hypermetabolic as well as nonhypermetabolic bone lesions. Based on these findings, the patient was referred for radiotherapy to the hypermetabolic bone lesions. After autologous peripheral blood stem cell transplantation, F-18 FDG PET/CT may play a pivotal role in detecting new bone lesions in patients with POEMS syndrome, which may be treated by a focalized radiotherapy and/or systemic therapy...
February 2012: Clinical Nuclear Medicine
Vaibhav Wadwekar, Jayantee Kalita, Usha Kant Misra
BACKGROUND: The clinical presentation, neurophysiological findings, and outcome may vary between primary and secondary chronic inflammatory demyelinating polyradiculopathy (CIDP). OBJECTIVE: To compare clinical and electrodiagnostic features of primary and secondary CIDP. SETTING: Tertiary care teaching referral hospital. MATERIALS AND METHODS: The CIDP patients who were diagnosed as per European Federation of Neurological Societies/Peripheral Nerve Society criteria were included and subjected to detailed history and examinations...
September 2011: Neurology India
Yukako Hattori, Jun Taguchi, Noboru Imai, Koichiro Wasano, Shiro Matsuura, Hideyuki Koharazawa, Yoshiaki Ishigatsubo, Shin Fujisawa
A 48-year-old male with Castleman disease developed symptoms typical of POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal-protein and skin changes) but M-protein was not detected by immunoelec-trophoresis. He was diagnosed as having POEMS syndrome based on IgA-λ detected by immunofixation electrophoresis and an increased level (3,170 pg/ml) of vascular endothelial growth factor (VEGF). After diagnosis, the patient underwent autologous peripheral blood stem cell transplantation safely and remained relapse-free and in good condition for 15 months...
April 2011: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
Jian Li, Wei Zhang, Li Jiao, Ming-Hui Duan, Hong-Zhi Guan, Wei-Guo Zhu, Zhuang Tian, Dao-Bin Zhou
POEMS syndrome is a rare clonal plasma cell disorder without standard treatment. Based on the efficacy and low toxicity of a combination of melphalan and dexamethasone (MDex) for light chain amyloidosis, we conducted a prospective study of MDex treatment for patients with newly diagnosed POEMS syndrome. Thirty-one patients (19 men) were enrolled and the median age at the time of diagnosis was 44 years (range, 32-68 years). All patients received 12 cycles of MDex treatment. Twenty-five patients (80.6%) achieved hematologic response including 12 (38...
June 16, 2011: Blood
Chiara Terracciano, Stefania Fiore, Elena Doldo, Vittorio Manzari, Girolama Alessandra Marfia, Giorgio Bernardi, Roberto Massa, Loredana Albonici
POEMS (polyneuropathy, organomegaly, endocrinopathy, M-band, and skin changes) syndrome is characterized by chronic progressive polyneuropathy and plasma-cell dyscrasia. A major diagnostic criterion of POEMS is elevation of circulating vascular endothelial growth factor (VEGF), which is believed to play a pathogenic role in this disease. We report a case of POEMS that presented as relapsing acute inflammatory demyelinating polyneuropathy, in which complete remission after intravenous immunoglobulin (IVIg) treatment was unexpectedly observed...
September 2010: Muscle & Nerve
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