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Myeloma, poems, transplantation

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...
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)
Carlo Rostagno, Stefania Ciolli, Domenico Prisco
No abstract text is available yet for this article.
2006: Internal and Emergency Medicine
Angela Dispenzieri
POEMS syndrome is defined by the presence of a peripheral neuropathy (P), a monoclonal plasma cell disorder (M), and other paraneoplastic features, the most common of which include organomegaly (O), endocrinopathy (E), skin changes (S), papilledema, edema, effusions, ascites, and thrombocytosis. Virtually all patients will have either sclerotic bone lesion(s) or co-existent Castleman's disease. Not all features of the disease are required to make the diagnosis, and early recognition is important to reduce morbidity...
2005: Hematology—the Education Program of the American Society of Hematology
Ilene B Bayer-Garner, Bruce R Smoller
BACKGROUND: Multiple myeloma (MM) is a plasma cell dyscrasia characterized by a clonal proliferation of plasma cells that produces a monoclonal protein. There are dermatologic disorders that have been associated with MM, such as amyloidosis, cryoglobulinemia, POEMS syndrome, normolipemic plane xanthoma, and plasmacytoma. The high volume of patients with MM seen at our institution presents an opportunity to define more extensively the spectrum of cutaneous diseases seen in concert with MM...
April 2003: Journal of the American Academy of Dermatology
W J Hogan, M Q Lacy, G A Wiseman, R D Fealey, A Dispenzieri, M A Gertz
POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes) is a plasma cell dyscrasia that differs substantially from classic multiple myeloma. It is often associated with disabling polyneuropathy in younger patients. Current therapeutic approaches are frequently inadequate and leave many patients wheelchair-bound with significant deterioration in quality and length of life. We present the case of a young man with progressive disease despite conventional therapeutic approaches...
August 2001: Bone Marrow Transplantation
Z Simmons
Paraprotein-associated neuropathies are a diverse group of disorders. The pathogenesis of many of them is poorly understood. Treatments have usually consisted of plasma exchange, corticosteroids, intravenous immunoglobulin, and other immunosuppressive therapies. Response to treatment has varied from good to very poor. Most recent work in this field has had two goals: achieving a better understanding of pathogenesis and developing better treatments. Such diverse entities as hepatitis C virus, vascular endothelial growth factor, and cytokines now appear to play a role in pathogenesis...
October 1999: Current Opinion in Neurology
M Ehrenfeld, H Gur, Y Shoenfeld
This review focuses on the various rheumatologic manifestations of hematologic diseases and is based on the English literature published over the past year. Interrelationships between leukemia, lymphoma, POEMS syndrome, multiple myeloma, hemophilia, and rheumatic manifestations are discussed. Other less common associated conditions dealt with include the RS3PE syndrome, large granular lymphocyte syndrome, and interferon-related arthritis. Finally, therapeutic measures, such as bone marrow transplantation and the use of granulocyte colony-stimulating factor, are reviewed...
January 1999: Current Opinion in Rheumatology
V A Wong, N K Wade
PURPOSE: To alert ophthalmologists to POEMS syndrome as an unusual cause of optic disk swelling. METHOD: Case report. A 25-year-old white woman developed scotomata and optic disk swelling, and systemic signs and symptoms of POEMS syndrome. RESULTS: Bone marrow biopsy disclosed multiple myeloma. Despite treatment with corticosteroids, radiation, and marrow transplantation, the patient died from multi-organ failure. CONCLUSION: Because POEMS syndrome typically affects older male patients, this is an unusual case of multiple myeloma in conjunction with POEMS syndrome...
September 1998: American Journal of Ophthalmology
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