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Myeloma, poems, transplantation

Sarah Ordway, Laura Gilbert, Sam Wanko
Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes (POEMS) syndrome is a rare systemic disease, often unrecognised in the primary care setting. POEMS syndrome is associated with plasma cell dyscrasias and upregulation of vascular endothelial growth factor leading to systemic oedema, papilloedema and pulmonary hypertension. A wide constellation of presenting symptoms often leads to late diagnosis. Unrecognised and untreated disease rapidly leads to death from neuropathic exhaustion or cardiopulmonary failure...
July 26, 2018: BMJ Case Reports
Maria Gavriatopoulou, Pellegrino Musto, Jo Caers, Giampaolo Merlini, Efstathios Kastritis, Niels van de Donk, Francesca Gay, Ute Hegenbart, Roman Hajek, Sonja Zweegman, Benedetto Bruno, Christian Straka, Meletios A Dimopoulos, Hermann Einsele, Mario Boccadoro, Pieter Sonneveld, Monika Engelhardt, Evangelos Terpos
The introduction of novel agents in the management of multiple myeloma and related plasma cell dyscrasias has changed our treatment approaches and subsequently the outcome of patients. Due to current advances, the European Myeloma Network updated the diagnostic and therapeutic recommendations for patients with Waldenström's macroglobulinemia (WM), AL-amyloidosis, monoclonal immunoglobulin deposition disease (MIDD), POEMS syndrome, and primary plasma cell leukemia. For patients with WM, the combination of rituximab with chemotherapy remains the treatment cornerstone, while the Bruton-tyrosine kinase inhibitor ibrutinib has been introduced and approved for relapsed/refractory disease...
September 2018: Leukemia: Official Journal of the Leukemia Society of America, Leukemia Research Fund, U.K
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
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
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...
January 2018: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
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
Aishwarya Ravindran, Ronald S Go, Fernando C Fervenza, Sanjeev Sethi
Thrombotic microangiopathy (TMA) is a rare disease comprising of a diverse set of disorders linked by a common histologic finding of endothelial injury. Monoclonal immunoglobulins may act as a potential trigger in the pathogenesis of TMA. To determine the prevalence of monoclonal gammopathy and clinicopathological features of TMA associated with monoclonal immunoglobulin, we performed a retrospective study in adults (18 and older) with a clinical diagnosis of TMA. Of 146 patients with TMA, we detected monoclonal immunoglobulin in 20 patients (13...
March 2017: Kidney International
Wayne L Feyereisn, Eric R Fenstad, Robert B McCully, Martha Q Lacy
An association between pulmonary hypertension (PH) and POEMS syndrome (characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) as well as other plasma cell dyscrasias, including multiple myeloma (MM), has been shown to exist. Recent case reports have identified a reversible form of PH that occurs outside of previously identified etiologies. We report two cases of PH in the setting of smoldering MM (SMM) that resolved with chemotherapy and stem cell transplantation...
March 2015: Pulmonary Circulation
Kanako Katayama, Sonoko Misawa, Yasunori Sato, Gen Sobue, Ichiro Yabe, Osamu Watanabe, Masatoyo Nishizawa, Susumu Kusunoki, Seiji Kikuchi, Ichiro Nakashima, Shu-Ichi Ikeda, Nobuo Kohara, Takashi Kanda, Jun-Ichi Kira, Hideki Hanaoka, Satoshi Kuwabara
INTRODUCTION: Polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes (POEMS) syndrome is a fatal systemic disorder associated with plasma cell dyscrasia and the overproduction of the vascular endothelial growth factor (VEGF). Recently, the prognosis of POEMS was substantially improved by introduction of therapeutic intervention for myeloma. However, no randomised clinical trial has been performed because of the rarity and severity of the disease. METHODS AND ANALYSIS: The Japanese POEMS syndrome with Thalidomide (J-POST) Trial is a phase II/III multicentre, double-blinded, randomised, controlled trial that aims to evaluate the efficacy and safety of a 24-week treatment with thalidomide in POEMS syndrome, with an additional 48-week open-label safety study...
January 8, 2015: BMJ Open
Yoshimi Ishii, Etsuko Yamazaki, Yasufumi Ishiyama, Eri Yamamoto, Yukako Hattori, Maki Hagihara, Naoto Tomita, Yoshiaki Ishigatsubo
POEMS syndrome is a monoclonal plasma cell disorder characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes. High-dose therapy (HDT) and autologous stem cell transplantation (ASCT) are an effective therapy, but optimal treatment options are still under debate. Bortezomib is an important agent for the treatment of patients with multiple myeloma and has recently been reported as efficacious in the treatment of patients with POEMS syndrome. We present a case of POEMS syndrome in a 33-year-old woman, who was successfully treated with BorDex (bortezomib and dexamethasone) combined with radiotherapy, and followed by ASCT...
December 2013: International Journal of Hematology
Alla Keyzner, Anita D'Souza, Martha Lacy, Morie Gertz, Suzanne Hayman, Francis Buadi, Shaji Kumar, David Dingli, Ann Engebretson, Caili Tong, Angela Dispenzieri
A rare, multisystem, plasma cell neoplasm, POEMS (polyradiculoneuropathy, organomegaly, endocrinopathy, M-spike, skin changes) syndrome is characterized by an abundance of proinflammatory and angiogenic cytokines. Patients with POEMS are known to have a high incidence of engraftment syndrome after autologous stem cell transplantation. We conducted a pilot study assessing levels of 30 different pro- and anti-inflammatory cytokines before and serially after transplantation in 18 patients with plasma cell neoplasms: POEMS syndrome (n = 9), multiple myeloma (n = 4), and amyloidosis (n = 5)...
September 2013: Biology of Blood and Marrow Transplantation
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
Ke Zeng, Jin-rong Yang, Jun Li, Qing Wei, Yi-ming Yang, Ting Liu, Ting Niu
POEMS syndrome is characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes. Bortezomib is an important component of the chemotherapy regimen associated with multiple myeloma, and has been previously applied to POEMS syndrome. We present a 56-year-old Chinese man who was given subcutaneous administration of bortezomib as part of the BDex (bortezomib-dexamethasone) regimen for his POEMS syndrome. The peripheral neuropathy and laboratory-test results of the patient improved dramatically with 4 cycles of treatment, resulting in a complete response...
2013: Acta Haematologica
Ronald C Walker, Tracy L Brown, Laurie B Jones-Jackson, Lorraine De Blanche, Twyla Bartel
Multiple myeloma (MM) is an incurable plasma cell malignancy of the bone marrow. MM has 3 components: diffuse marrow infiltration, focal bone lesions, and soft-tissue (extramedullary) disease. The hallmark biomarker in blood or urine is a monoclonal immunoglobulin, the monoclonal protein. Waldenstrom macroglobulinemia is a similar disease with secretion of IgM. Staging is classically performed with the 1975 Durie-Salmon system, which includes conventional radiographs. Recently updated, the Durie-Salmon Plus staging system includes CT, MRI, and (18)F-FDG PET/CT...
July 2012: Journal of Nuclear Medicine: Official Publication, Society of Nuclear Medicine
Anita D'Souza, Martha Lacy, Morie Gertz, Shaji Kumar, Francis Buadi, Suzanne Hayman, David Dingli, Steven Zeldenrust, Robert Kyle, Stephen Ansell, David Inwards, Patrick Johnston, Ivana Micallef, Luis Porrata, Mark Litzow, Dennis Gastineau, William Hogan, Angela Dispenzieri
The POEMS syndrome (polyradiculoneuropathy, organomegaly, multiple endocrinopathies, monoclonal protein, skin changes) is a rare disease associated with a plasma cell dyscrasia. Patients with disseminated POEMS can be treated with high-dose therapy and autologous stem cell transplantation (ASCT). While clinical improvement is nearly universal in these patients, the long-term outcomes after transplantation are unclear. We therefore assessed the long-term clinical outcomes of 59 POEMS patients treated with ASCT at our institution...
July 5, 2012: Blood
Carlos R Méndez-Herrera, Damisela Cordoví-Rodríguez
POEMS syndrome is a rare multisystemic paraneoplastic disorder. That is associated with a plasma cell dyscrasia. It is characterized by peripheral neuropathy, organomegaly, endocrinopathy, M protein, skin changes and other systemic features. The pathogenesis of POEMS is not well understood, but overproduction of vascular endothelial growth factor is likely to be responsible for most of characteristic symptoms. There are no randomized controlled trials in patients with POEMS syndrome but are therapies which benefit these patients, including radiation therapy, chemotherapy, cortico-steroids and stem cell transplant...
July 1, 2011: Revista de Neurologia
Hiroshi Iwashita
In 1971, the author reported an autopsy case of a 48-year-old Japanese man with polyneuropathy, skin hyperpigmentation, diabetes mellitus, and monoclonal gammopathy. Previously, a total of 2 cases of solitary myeloma accompanied by polyneuropathy and endocrinological disorders have been reported by Fukase et al in 1968 and by Shimomori and Kusumoto in 1970 in Japan. The author's case is the first reported non-myeloma case where polyneuropathy associated with dermatoendocrionological changes and dysglobulinemia was observed...
February 2011: Brain and Nerve, Shinkei Kenkyū No Shinpo
Jebin M Chacko, Warren D Spinner
Paraproteinemic neuropathies comprise a diverse group of disorders that includes monoclonal gammopathy of undetermined significance, primary amyloidosis, multiple myeloma, cryoglobulinemia, POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal protein and skin changes) syndrome, and Waldenstrom macroglobulinemia. Various factors including hepatitis C virus, vascular endothelial growth factor, and an array of cytokines are implicated in the pathogenesis of these conditions. More recently, a variety of novel antibody specificities, and vasculitis, have also been described as contributory factors in the development of these neuropathies...
June 2006: Journal of Clinical Neuromuscular Disease
Ciğdem A Akkök, Mette R Holte, Jon M Tangen, Bjørn Ostenstad, Oystein Bruserud
BACKGROUND: Autologous stem cell transplantation with cryopreserved autografts is a prerequisite for high-dose chemotherapy in treatment of several malignancies. Adverse effects due to the cryoprotectant dimethyl sulfoxide (DMSO) vary from mild to severe. DMSO-associated adverse effects can be reduced by DMSO depletion before autograft infusion. The aim was to investigate whether DMSO depletion by manual single wash reduced frequency of adverse effects or had detrimental effects on the engraftment potential of peripheral blood progenitor cell (PBPC) autografts...
February 2009: Transfusion
Sachin Gupta, Vishal Rana, Divay Chandra, Sergio A Giralt, Harinder S Juneja
Hematopoietic stem cell transplant has been used in the treatment of multiple myeloma. Its use in the treatment of POEMS syndrome is still under investigation. We describe the clinical course and long term follow-up of a patient with POEMS syndrome unresponsive to standard chemotherapy. The patient had rapid and dramatic improvement in neuropathy and dermatologic features after autologous stem cell transplantation. A review of the literature is also presented. High-dose chemotherapy followed by stem cell transplantation is a potential treatment option for patients with POEMS syndrome...
October 2006: Hematology (Amsterdam, Netherlands)
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