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Monoclonal gammopathy

C Lartigau-Roussin, M H Paclet, H Audin, B Toussaint, T Henni, L Raffray
INTRODUCTION: Macro-AST is recognized as a classical aetiology of isolated and persistent increase of serum aspartate aminotransferase (AST) levels. Macro-AST are high molecular weight complexes associating AST and a macromolecule, often an immunoglobulin. Although those macroenzymes of unknown pathogenesis are usually non-pathogenic, association with several diseases, including autoimmune diseases and liver diseases has been described. CASE REPORT: We report here the case of a 45-year-old patient with previously normal liver enzymes in whom an AST elevation and an IgA monoclonal gammopathy were discovered concomitantly...
October 13, 2016: La Revue de Médecine Interne
Madhav V Dhodapkar
All cases of multiple myeloma (MM) are preceded by precursor states termed as monoclonal gammopathy of undetermined significance (MGUS) or smoldering myeloma (SMM). Genetic analyses of MGUS cells have provided evidence that it is a genetically advanced lesion wherein tumor cells carry many of the genetic changes found in MM cells. Intraclonal heterogeneity is also established early during the MGUS phase. While the genetic features of MGUS or SMM cells at baseline may predict disease risk, transition to MM involves altered growth of pre-existing clones...
October 13, 2016: Blood
E Terpos, D Christoulas, E Kastritis, T Bagratuni, M Gavriatopoulou, M Roussou, A Papatheodorou, E Eleutherakis-Papaiakovou, N Kanellias, C Liakou, I Panagiotidis, M Migkou, P Kokkoris, L A Moulopoulos, M A Dimopoulos
Periostin is an extracellular matrix protein that is implicated in the biology of normal bone remodeling and in different cancer cell growth and metastasis. However, there is no information on the role of periostin in multiple myeloma (MM). Thus, we evaluated periostin in six myeloma cell lines in vitro; in the bone marrow plasma and serum of 105 newly diagnosed symptomatic MM (NDMM) patients and in the serum of 23 monoclonal gammopathy of undetermined significance (MGUS), 33 smoldering MM (SMM) patients, 30 patients at the plateau phase post-first-line therapy, 30 patients at first relapse and 30 healthy controls...
October 7, 2016: Blood Cancer Journal
Zdeněk Adam, Anna Šedivá, Renata Koukalová, Zdeněk Řehák, Hana Petrášová, Petr Szturz, Zdenka Adamová, Eva Vetešníková, Luděk Pour, Marta Krejčí, Viera Sandecká, Eva Pourová, Zdeňka Čermáková, Sabina Ševčíková, Zdeněk Král, Jiří Mayer
Schnitzlers syndrome is an acquired auto-inflammatory disease of still unclear origin. The Strasbourg criteria were adopted (non-infectious fever, chronic urticaria, changes in the bone structure, leukocytosis and higher values of inflammatory markers - CRP and presence of monoclonal immunoglobulin mostly of type IgM, very rarely of IgG) to establish this diagnosis. The first-choice therapy for this disease is the blocking of interleukin-1 effects. In practice, the interleukin-1 receptor antagonist, anakinra, is the most commonly used...
2016: Vnitr̆ní Lékar̆ství
Chirawadee Sathitruangsak, Christiaan H Righolt, Ludger Klewes, Doris Tung Chang, Rami Kotb, Sabine Mai
The consistent appearance of specific chromosomal translocations in multiple myeloma has suggested that the positioning of chromosomes in the interphase nucleus might play a role in the occurrence of particular chromosomal rearrangements associated with malignant transformation. Using fluorescence in situ hybridization, we have determined the positions of selected chromosome pairs (18 and 19, 9 and 22, 4 and 14, 14 and 16, 11 and 14) in interphase nuclei of myeloma cells compared to normal lymphocytes of treatment-naïve patients...
October 6, 2016: International Journal of Cancer. Journal International du Cancer
Melike Kalfa, Hayriye Koçanaoğulları, Figen Yargucu Zihni, Gonca Karabulut, Hakan Emmungil, Vedat İnal
Scleredema is a rare connective tissue disorder of unknown pathogenesis. Three types of scleredema have been described, based on its association with postinfection, monoclonal gammopathy and diabetes mellitus. We report herein a case of scleredema which the diagnosis didn't get specified. The patient was followed regularly for 13 years and did not respond to various combinations of immunosuppressants and psoralen plus ultraviolet A therapy. Treatment of scleredema is quite difficult and of limited success. At present, there is no proved treatment for this disease...
December 2015: Eur J Rheumatol
Yusuf Savran, Sevgi Akarsu
Scleromyxedema is an uncommon connective tissue disease characterized by mucin deposits, fibrosis, and proliferation of fibroblasts in the dermis. Although it shares similar sclerodermoid features, it is a different clinical entity than scleroderma. A monoclonal gammopathy is almost always present; however, progression to multiple myeloma is rare. It may have many systemic manifestations, of which the most notable being the dermato-neuro syndrome because of its rarity and potential fatal outcome. We present a case of a 50-year-old woman with scleromyxedema in whom the dermato-neuro syndrome developed...
December 2015: Eur J Rheumatol
Hirotaka Yokouchi, Takayuki Baba, Sonoko Misawa, Masayasu Kitahashi, Toshiyuki Oshitari, Satoshi Kuwabara, Shuichi Yamamoto
AIMS: To determine the changes in the subfoveal choroidal thickness (CT), the foveal thickness (FT) and the serum level of vascular endothelial growth factor (VEGF) after thalidomide treatment in patients with polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes (POEMS) syndrome. METHODS: We studied 13 left eyes of 13 treatment-naïve patients with POEMS syndrome. The subfoveal CT and FT were determined by enhanced depth imaging optical coherence tomography, and the serum level of VEGF was determined by ELISA at the baseline and at 6 months after thalidomide treatment...
October 4, 2016: British Journal of Ophthalmology
Natalia Ermak, Thao Nguyen-Khoa, Marie-Alexandra Alyanakian
The diagnostics and follow-up of monoclonal gammopathies such as multiple myeloma require precise analysis of the monoclonal component as well as the other immunoglobulins isotypes, which might be limited by the sensitivity of standard laboratory methods. New serum biomarkers were developed for routine practice in the last decades, such as the free light chain assays and more recently the heavy/light chain assay<strike>s</strike>. Studies have shown that serum free light chain measurement was useful in the identification and follow-up of pauci or nonsecretory myeloma, free light-chain multiple myeloma and AL amyloidosis...
October 1, 2016: Annales de Biologie Clinique
Michael Pt Lunn, Eduardo Nobile-Orazio
BACKGROUND: Serum monoclonal anti-myelin-associated glycoprotein (anti-MAG) antibodies may be pathogenic in some people with immunoglobulin M (IgM) paraprotein and demyelinating neuropathy. Immunotherapies aimed at reducing the level of these antibodies might be expected to be beneficial. This is an update of a review first published in 2003 and previously updated in 2006 and 2012. OBJECTIVES: To assess the effects of immunotherapy for IgM anti-MAG paraprotein-associated demyelinating peripheral neuropathy...
October 4, 2016: Cochrane Database of Systematic Reviews
Alfredo Gagliardi, Claudio Carbone, Angela Russo, Rosanna Cuccurullo, Anna Lucania, Paola Della Cioppa, Gabriella Misso, Michele Caraglia, Catello Tommasino, Lucia Mastrullo
Monoclonal gammopathies are characterized by serum monoclonal component (MC) plus an intact immunoglobulin and a free light chain (FLC), or a combination of both. The measurement of FLC with Freelite(®) is the standard practice recommended by International Myeloma Working Group guidelines. Recently, Hevylite(®) heavy/light chains (HLC) assays were introduced to specifically target junctional epitopes between the heavy and light chains of intact immunoglobulins, allowing the independent quantification of the involved (MC) and uninvolved (polyclonal immunoglobulin background) HLC isotype...
October 2016: Oncology Letters
Sue-Ann Teh, David A Kandiah
Scleromyxedema is part of a group of cutaneous mucinoses, characterized by a generalized papular eruption, dermal mucin deposition, and an increase in dermal collagen. This condition can be localized as discrete papular lichen myxedematous skin or as a systemic condition usually associated with paraproteinaemia. To date, there is no unifying treatment and is limited by rarity, small number of case reports, and the lack of randomized controlled trials. We describe the case of a 56-year-old gentleman with features of scleromyxedema who had cutaneous and cardiac involvement, and significant mediastinal lymphadenopathy without monoclonal gammopathy...
2016: International Medical Case Reports Journal
Chen Wang, Xu-Fei Huang, Qian-Qian Cai, Xin-Xin Cao, Hao Cai, Daobin Zhou, Jian Li
Vascular endothelial growth factor (VEGF) is pathognomonically elevated in patients with POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes) syndrome. However, its source of overproduction is unclear. As clinical improvement is almost always associated with VEGF reduction after anti-plasma cell therapy, its increase at diagnosis has been attributed to the underlying monoclonal gammopathy, although direct evidence is still lacking. In the current study, we systemically measured VEGF levels in POEMS patients, before and after treatment...
September 26, 2016: Leukemia Research
Domenico Ribatti, Angelo Vacca
Multiple myeloma (MM) mainly progresses in bone marrow (BM). Therefore, signals from the BM microenvironment are thought to play a critical role in maintaining plasma cell growth, migration, and survival. Reciprocal positive and negative interactions between plasma cells and microenvironmental cells, including endothelial cells (ECs) and fibroblasts may occur. The BM neovascularization is a constant hallmark of MM, and goes hand in hand with progression to leukemic phase. Microenvironmental factors induce MMECs and fibroblasts to become functionally different from monoclonal gammopathy of undetermined significance (MGUS) ECs (MGECs), i...
2016: Cancer Treatment and Research
María-Victoria Mateos, Ola Landgren
Monoclonal gammopathy of undetermined significance (MHUS) is characterized by the presence of a serum M-protein less than 3 g/dL, less than 10 % clonal plasma cells in the bone marrow, and the absence of myeloma-defining event. Smoldering multiple myeloma (SMM) is an asymptomatic disorder characterized by the presence of ≥3 g/dL serum M-protein and/or 10-60 % bone marrow plasma cell infiltration with no myeloma-defining event. The risk of progression to multiple myeloma (MM) requiring therapy varies greatly for individual patients, but it is uniform and 1 % per year for MGUS, while higher (10 % per year) and not uniform for SMM patients...
2016: Cancer Treatment and Research
Federica Arginelli, Franco Rongioletti, Giampiero Girolomoni, Giovanni Pellacani, Davide Guardoli, Andrea Conti
Scleromyxoedema is a rare disease with a progressive and disabling course involving dermal deposition of mucin and fibroblast proliferation; it is characterized clinically by a diffuse papular eruption, skin thickening, oedema and decreased skin flexibility, especially of the face and hands. Current therapy options are based on evidence from a limited number of case reports. The clinical manifestations and treatment of a 64-year-old man affected by scleromyxoedema with severe skin involvement of the face, arms and hands, decreased mouth opening and hypomotility of the fingers are reported...
September 2016: Journal of International Medical Research
Karoline Krause, Athanasios Tsianakas, Nicola Wagner, Jörg Fischer, Karsten Weller, Martin Metz, Martin K Church, Marcus Maurer
BACKGROUND: Schnitzler's syndrome is an adult-onset autoinflammatory disease characterized by urticarial exanthema and monoclonal gammopathy accompanied by systemic symptoms such as fever, bone and muscle pain. Up to now, approved treatment options are not available. OBJECTIVE: We here assessed the effects of the anti-IL-1ß monoclonal antibody canakinumab on the clinical signs and symptoms of Schnitzler's syndrome. METHODS: In this phase II, randomized placebo-controlled multi-center study, 20 patients with active disease enrolled in four German study centers...
September 19, 2016: Journal of Allergy and Clinical Immunology
John Gubatan, Xiaohui Wang, Abner Louissaint, Anuj Mahindra, John Vanderpool
BACKGROUND: Bone marrow sarcoidosis is extremely rare. The association between sarcoidosis and lymphoproliferative disorders has been previously speculated, although the diagnosis of sarcoidosis often precedes any hematological derangements. CASE PRESENTATION: Here, we report for the first time, a case of a 57-year-old Caucasian woman with a previous diagnosis of monoclonal gammopathy of undetermined significance (MGUS) developing hypercalcemia and renal failure with workup notable for isolated bone marrow sarcoidosis and not multiple myeloma as expected...
2016: Biomarker Research
Benedetta Miglino, Michele Viana, Rossana Tiberio, Paolo Boggio, Guido Valente, Gionata Strigaro, Enrico Colombo
The authors report a case of sensorimotor polyneuropathy, diffuse hemangiomas and monoclonal gammopathy. Besides weight loss, there were diabetes mellitus and severe hypothyroidism. These alterations were consistent with POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal gammopathy and Skin changes) syndrome, which is a rare systemic disease with monoclonal proliferation of plasmacytes and slow progression. Because of its rarity, the incidence of this disease is still unknown.
September 15, 2016: Giornale Italiano di Dermatologia e Venereologia: Organo Ufficiale, Società Italiana di Dermatologia e Sifilografia
Catarina Geraldes, Ana Cristina Gonçalves, Emília Cortesão, Marta Isabel Pereira, Adriana Roque, Artur Paiva, Letícia Ribeiro, José Manuel Nascimento-Costa, Ana Bela Sarmento-Ribeiro
BACKGROUND: Aberrant DNA methylation is considered a crucial mechanism in the pathogenesis of monoclonal gammopathies. We aimed to investigate the contribution of hypermethylation of 4 tumor suppressor genes to the multistep process of myelomagenesis. METHODS: The methylation status of p15, p16, p53, and DAPK genes was evaluated in bone marrow samples from 94 patients at diagnosis: monoclonal gammopathy of uncertain significance (MGUS) (n = 48), smoldering multiple myeloma (SMM) (n = 8) and symptomatic multiple myeloma (MM) (n = 38), and from 8 healthy controls by methylation-specific polymerase chain reaction analysis...
August 10, 2016: Clinical Lymphoma, Myeloma & Leukemia
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