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https://www.readbyqxmd.com/read/28512563/acquired-von-willebrand-syndrome-associated-to-secondary-igm-mgus-emerging-after-autologous-stem-cell-transplantation-for-al-amyloidosis
#1
Hina Qamar, Adrienne Lee, Karen Valentine, Leslie Skeith, Victor H Jimenez-Zepeda
Acquired von Willebrand syndrome (AVWS) is a rare hemorrhagic disorder that occurs in patients with no prior personal or family history of bleeding. Here, we describe a case of AVWS occurring after autologous stem cell transplantation (ASCT). Interestingly, AVWS developed after bortezomib-based induction and conditioning regimens. Recent evidence suggests that the proximity of the bortezomib therapy to the collection of stem cells with consequent depletion of regulatory T cells after the conditioning regimen could explain some of the unusual autoimmune complications reported in patients receiving bortezomib prior to ASCT...
2017: Mediterranean Journal of Hematology and Infectious Diseases
https://www.readbyqxmd.com/read/28397326/diagnosis-and-management-of-neuropathies-associated-with-plasma-cell-dyscrasias
#2
REVIEW
Evan Rosenbaum, Douglas Marks, Shahzad Raza
Neuropathies associated with plasma cell dyscrasias are a major cause of morbidity for patients managed by medical oncologists. Because of similarities in clinical presentation and on nerve conduction studies, identifying the underlying disease leading to a paraproteinemic neuropathy can often be difficult. In addition, the degree of neurologic deficit does not strictly correlate with the extent of abnormalities on common clinical laboratory testing. Fortunately, with increasing understanding into the biologic mechanisms of underlying hematologic diseases, additional biomarkers have recently been developed, thus improving our diagnostic capacity...
April 10, 2017: Hematological Oncology
https://www.readbyqxmd.com/read/28284031/immunoparesis-in-igm-gammopathies-as-a-useful-biomarker-to-predict-disease-progression
#3
Marcio Andrade-Campos, Ilda Murillo-Flórez, Ramón García-Sanz, Pilar Giraldo
BACKGROUND: The management of IgM monoclonal gammopathies undetermined significance (IgM-MGUS) and Waldenstrom's macroglobulinemia (WM) may be challenging. Modern immunoassays that quantify specific monoclonal heavy and light chain immunoglobulins are promising for their use in these applications. METHODS: Ninety consecutive patients (39 IgM-MGUS, 32 indolent WM [iWM], and 19 WM) seen between January 2007 and March 2014 were analyzed. Heavy/light chain (HLC) and serum free light chains assays (FLC) were determined at diagnosis to study their utility as biomarkers in IgM monoclonal gammopathies...
March 11, 2017: Clinical Chemistry and Laboratory Medicine: CCLM
https://www.readbyqxmd.com/read/28280994/detection-of-myd88-l265p-and-whim-like-cxcr4-mutation-in-patients-with-igm-monoclonal-gammopathy-related-disease
#4
Xin-Xin Cao, Qi Meng, Hao Cai, Tian-Hua He, Cong-Li Zhang, Wei Su, Jian Sun, Yue Li, Wei Xu, Dao-Bin Zhou, Jian Li
A broad spectrum of diseases are associated with IgM monoclonal gammopathy, including Waldenstrom macroglobulinemia (WM), various types of B cell non-Hodgkin's lymphoma (NHL), multiple myeloma (MM), primary amyloidosis (AL), and monoclonal gammopathy of undetermined significance (MGUS); these are called IgM monoclonal gammopathy related diseases (IgM-RD). We investigated MYD88 L265P and WHIM-like CXCR4 mutations in various IgM-RD. Patients with serum immunofixation electrophoresis confirmed IgM monoclonal gammopathy who had enough material for DNA extraction and presented between January 2008 and October 2016 at Peking Union Medical College Hospital were enrolled in this cohort...
March 9, 2017: Annals of Hematology
https://www.readbyqxmd.com/read/27974705/are-neurological-complications-of-monoclonal-gammopathy-of-undetermined-significance-underestimated
#5
Normann Steiner, Angelika Schwärzler, Georg Göbel, Wolfgang Löscher, Julia Wanschitz, Eberhard Gunsilius
OBJECTIVES: Monoclonal gammopathy of undetermined significance (MGUS) is a premalignancy preceding multiple myeloma (MM) or related disorders. Neurological symptoms caused by the monoclonal immunoglobulins or free light-chains are often associated with a high morbidity. We analyzed the prevalence of neuropathy, clinical features and the long-term outcome in 223 patients (pts.) with MGUS. PATIENTS AND METHODS: Between 1/2005 and 3/2015, 223 adult pts. with MGUS were identified in our database...
January 17, 2017: Oncotarget
https://www.readbyqxmd.com/read/27825465/monoclonal-gammopathy-of-undetermined-significance-and-waldenstr%C3%A3-m-s-macroglobulinemia
#6
REVIEW
Sham Mailankody, Ola Landgren
Since the initial identification of patients with monoclonal elevation of gamma globulin and associated clinical symptoms in 1944 by Jan Waldenström, several new insights have been gained using a range of approaches. For example, IgM monoclonal gammopathy of undetermined significance and smoldering Waldenström's macroglobulinemia are defined clinical precursor states to symptomatic Waldenström's macroglobulinemia. Epidemiologic studies have established the prevalence of these conditions and the estimated risk of progression to symptomatic disease...
June 2016: Best Practice & Research. Clinical Haematology
https://www.readbyqxmd.com/read/27580763/a-case-of-severe-chronic-inflammatory-demyelinating-polyradiculoneuropathy-with-monoclonal-gammopathy-of-undetermined-significance-with-alternating-immunoglobulin-class-to-igm-from-iga
#7
Shintaro Hayashi, Shun Nagamine, Kouki Makioka, Susumu Kusunoki, Koichi Okamoto
A 71-year-old woman with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) with IgA-λ monoclonal gammopathy of undetermined significance (MGUS) showed the acute development of tetraplegia, respiratory failure, and a marked fluctuation of the blood pressure. Intravenous (IV) high-dose steroid therapy (methylprednisolone: 1 g/day × 3 days), followed by oral prednisolone (PSL) (40 mg/day), and IV immunoglobulin (IVIg, 0.4 g/kg/day × 5 days) administrations resulted in the amelioration of these symptoms...
September 29, 2016: Rinshō Shinkeigaku, Clinical Neurology
https://www.readbyqxmd.com/read/27282561/persistent-immune-alterations-and-comorbidities-in-splenectomized-patients-with-gaucher-disease
#8
Søren Ulrik Sønder, Renuka Pudi Limgala, Margarita M Ivanova, Chidima Ioanou, Matthew Plassmeyer, Gerald E Marti, Oral Alpan, Ozlem Goker-Alpan
Gaucher disease (GD) is an autosomal recessive disorder caused by mutations in the gene encoding acid-β-glucosidase, resulting in functional disruptions in degradation of glycosphingolipids and lysosomal accumulation of the substrates. The most frequent clinical presentations of GD are thrombocytopenia, splenomegaly and bone pain. Prior to advent of enzyme replacement therapy, splenectomy was performed for complications of hypersplenism such as severe thrombocytopenia and transfusion dependency. Though there is evidence about worsening bone disease after splenectomy, there is no systematic study to assess its effects on the immune system in GD patients...
July 2016: Blood Cells, Molecules & Diseases
https://www.readbyqxmd.com/read/27232065/the-clinical-spectrum-of-igm-monoclonal-gammopathy-a-single-center-retrospective-study-of-377-patients
#9
Xin-Xin Cao, Qi Meng, Yue-Ying Mao, Wei Su, Jun-Feng Zhen, Kai-Ni Shen, Chun-Lan Zhang, Xu-Fei Huang, Ming-Hui Duan, Wei Zhang, Tie-Nan Zhu, Hua-Cong Cai, Miao Chen, Dao-Bin Zhou, Jian Li
OBJECTIVES: We retrospectively evaluated the clinical features, serum levels of IgM, and prevalence of IgM related diseases in patients with serum immunofixation electrophoresis (sIFE) confirmed IgM monoclonal gammopathy at our center. METHODS: We included patients with sIFE confirmed IgM monoclonal gammopathy between January 2008 and December 2014 in this retrospective study. We evaluated clinical data, sIFE, serum IgM levels, and diagnosis. RESULTS: In total, 7107 patients had sIFE confirmed monoclonal gammopathy, with 377 (5...
July 2016: Leukemia Research
https://www.readbyqxmd.com/read/27188752/long-term-disability-and-prognostic-factors-in-polyneuropathy-associated-with-anti-myelin-associated-glycoprotein-mag-antibodies
#10
Giuliana Galassi, Manuela Tondelli, Alessandra Ariatti, Francesca Benuzzi, Paolo Nichelli, Franco Valzania
AIM OF THE STUDY: Neuropathy associated with IgM monoclonal gammopathy (MGUS) represents distinctive clinical syndrome, characterized by male predominance, late age of onset, slow progression, predominantly sensory symptoms, deep sensory loss, ataxia, minor motor impairment. More than 50% of patients with neuropathy-associated MGUS possess antibodies against myelin-associated glycoprotein (MAG). Purpose of our study was to assess effects on disease progression of demographic, clinical and neurophysiological variables in our large cohort of patients...
May 2017: International Journal of Neuroscience
https://www.readbyqxmd.com/read/27153790/state-of-the-art-imaging-and-staging-of-plasma-cell-dyscrasias
#11
REVIEW
Behrang Amini, Sarvari Yellapragada, Shetal Shah, Eric Rohren, Raghunandan Vikram
Monoclonal gammopathy of unknown significance (MGUS) is a clinically asymptomatic premalignant clonal plasma cell or lymphoplasmacytic proliferative disorder. Smoldering multiple myeloma, also called asymptomatic multiple myeloma, is an intermediate stage between MGUS and symptomatic multiple myeloma. As the name implies, extraosseous or extramedullary myeloma refers to the presence of myeloma deposits outside the skeletal system. Waldenström macroglobulinemia is a distinct subtype of plasma cell dyscrasia characterized by lymphoplasmacytic lymphoma in the bone marrow with an associated IgM monoclonal gammopathy...
May 2016: Radiologic Clinics of North America
https://www.readbyqxmd.com/read/27065581/analyzing-relationship-between-monoclonal-gammopathy-of-undetermined-significance-mgus-with-different-types-of-neuropathy-an-observational-study
#12
Shahzaib Nabi, Pushpinderdeep Kahlon, Farshid Bozorgnia, Adeel Arshad, Akmam Saleem, Philip Kuriakose
To analyze multiple variables, including immunoglobulin subtypes in patients with monoclonal gammopathy of undetermined significance (MGUS) and different types of neuropathy. This was a retrospective, single center study done in a tertiary care hospital in the United States. The data was collected for years 2001-2011. Inclusion criteria were the presence of MGUS and neuropathy. Exclusion criteria were the presence of other factors such as diabetes, vitamin B12 deficiency, alcoholism etc. which can cause neuropathy...
June 2016: Indian Journal of Hematology & Blood Transfusion
https://www.readbyqxmd.com/read/27040683/distinct-mechanisms-account-for-acquired-von-willebrand-syndrome-in-plasma-cell-dyscrasias
#13
Christina Dicke, Sonja Schneppenheim, Katharina Holstein, Brigitte Spath, Carsten Bokemeyer, Rita Dittmer, Ulrich Budde, Florian Langer
Acquired von Willebrand syndrome (AVWS) is a rare bleeding disorder that may cause life-threatening hemorrhages in patients with plasma cell dyscrasias (PCDs). Early diagnosis and treatment require a thorough understanding of its underlying pathophysiology. Two patients with IgG MGUS presented with dramatically decreased plasma von Willebrand factor (VWF) and a severe type-1 pattern on multimer analysis. A prompt response to intravenous immunoglobulins (IVIG), but not to VWF/FVIII, was consistent with accelerated immunologic clearance of plasma VWF...
May 2016: Annals of Hematology
https://www.readbyqxmd.com/read/27013181/prognostic-impact-of-serum-heavy-light-chain-pairs-in-patients-with-monoclonal-gammopathy-of-undetermined-significance-and-smoldering-myeloma-long-term-results-from-a-single-institution
#14
Laura Magnano, Carlos Fernández de Larrea, Montserrat Elena, María Teresa Cibeira, Natalia Tovar, Juan I Aróstegui, Fabiola Pedrosa, Laura Rosiñol, Xavier Filella, Jordi Yagüe, Joan Bladé
BACKGROUND: Asymptomatic monoclonal gammopathies, such as monoclonal gammopathy of undetermined significance (MGUS) and smoldering myeloma (SMM), are clinical conditions that usually precede symptomatic multiple myeloma. Therefore, risk stratification is crucial owing to the heterogeneous progression rate among these patients. In previous years, suppression of the uninvolved chain of specific heavy/light chain (HLC) pairs in serum has been identified as a new risk factor in MGUS. The aim of the present study was to investigate the prognostic effect of involved and uninvolved HLC pairs and HLC ratios on progression in a series of patients with MGUS and SMM...
June 2016: Clinical Lymphoma, Myeloma & Leukemia
https://www.readbyqxmd.com/read/26912783/poor-correlation-between-pneumococcal-igg-and-igm-titers-and-opsonophagocytic-activity-in-vaccinated-patients-with-multiple-myeloma-and-waldenstrom-s-macroglobulinemia
#15
Johanna Karlsson, Lucy Roalfe, Harriet Hogevik, Marta Zancolli, Björn Andréasson, David Goldblatt, Christine Wennerås
Patients with multiple myeloma and other B cell disorders respond poorly to pneumococcal vaccination. Vaccine responsiveness is commonly determined by measuring pneumococcal serotype-specific antibodies by enzyme-linked immunosorbent assay (ELISA), by a functional opsonophagocytosis assay (OPA), or by both assays. We compared the two methods in vaccinated elderly patients with multiple myeloma, Waldenstrom's macroglobulinemia, and monoclonal gammopathy of undetermined significance (MGUS). Postvaccination sera from 45 patients (n= 15 from each patient group) and 15 control subjects were analyzed by multiplexed OPA for pneumococcal serotypes 4, 6B, 14, and 23F, and the results were compared to IgG and IgM antibody titers measured by ELISA...
April 2016: Clinical and Vaccine Immunology: CVI
https://www.readbyqxmd.com/read/26909911/expression-and-significance-of-mir-21-in-multiple-myeloma-patients
#16
J H Wang, W W Zhou, B X Liu, D L Man, Z D Yang, F R Liu, H Shang
The aim of the present study is to examine the expression level of peripheral mir-21 in multiple myeloma (MM) patients and to determine its clinical significance. MM patients (30), monoclonal gammopathy of undetermined significance (MGUS) patients (14), and normal controls (20) were recruited to determine the serum level of β2-MG, IgA and IgM, IgG, λ, κ, TP, ALB, Hb, LDH, and Ca(2+). Gene expression of mir-21 was quantified by SYBR green real-time fluorescent quantitative PCR. We found that the expression level of serum mir-21 in the MM group was significantly higher than the MGUS group and the NC group (P < 0...
January 22, 2016: Genetics and Molecular Research: GMR
https://www.readbyqxmd.com/read/26797804/positive-selection-and-high-sensitivity-test-for-myd88-mutations-using-locked-nucleic-acid
#17
A Albitar, W Ma, I DeDios, J Estella, S Agersborg, M Albitar
INTRODUCTION: Detection of mutations in the myeloid differentiation primary response gene 88 (MYD88) has clinical implications on diagnosis and therapy, especially in patients with Waldenström's macroglobulinemia (WM) and IgM monoclonal gammopathy of unknown significance (IgM-MGUS). We describe a method that provides greatly increased sensitivity for detecting minority mutations in MYD88. METHODS: We used a locked nucleic acid oligonucleotide to block amplification of wild-type DNA during polymerase chain reaction (PCR)...
April 2016: International Journal of Laboratory Hematology
https://www.readbyqxmd.com/read/26659815/clonal-architecture-of-cxcr4-whim-like-mutations-in-waldenstr%C3%A3-m-macroglobulinaemia
#18
Lian Xu, Zachary R Hunter, Nicholas Tsakmaklis, Yang Cao, Guang Yang, Jie Chen, Xia Liu, Sandra Kanan, Jorge J Castillo, Yu-Tzu Tai, James L Zehnder, Jennifer R Brown, Ruben D Carrasco, Ranjana Advani, Jean M Sabile, Kimon Argyropoulos, M Lia Palomba, Enrica Morra, Alessandra Trojani, Antonino Greco, Alessandra Tedeschi, Marzia Varettoni, Luca Arcaini, Nikhil M Munshi, Kenneth C Anderson, Steven P Treon
CXCR4(WHIM) somatic mutations are distinctive to Waldenström Macroglobulinaemia (WM), and impact disease presentation and treatment outcome. The clonal architecture of CXCR4(WHIM) mutations remains to be delineated. We developed highly sensitive allele-specific polymerase chain reaction (AS-PCR) assays for detecting the most common CXCR4(WHIM) mutations (CXCR4(S338X C>A and C>G) ) in WM. The AS-PCR assays detected CXCR4(S338X) mutations in WM and IgM monoclonal gammopathy of unknown significance (MGUS) patients not revealed by Sanger sequencing...
March 2016: British Journal of Haematology
https://www.readbyqxmd.com/read/26559865/familial-waldenstrom-s-macroglobulinemia-and-relation-to-immune-defects-autoimmune-diseases-and-haematological-malignancies-a-population-based-study-from-northern-sweden
#19
Lena Brandefors, Eva Kimby, Kristina Lundqvist, Beatrice Melin, Jack Lindh
BACKGROUND: Waldenstrom's macroglobulinemia (WM) is a rare lymphoprolipherative disorder with geographic and ethnic disparities in incidence. The cause of WM remains mostly unknown although a role for genetic, immune-related, and environmental factors has been suggested. Most cases of WM are sporadic although familial cases occur. AIM: This study estimated the incidence of WM in northern Sweden and identified and described patients with familial WM in this area...
2016: Acta Oncologica
https://www.readbyqxmd.com/read/26484033/fibrillary-glomerulonephritis-combined-with-chronic-inflammatory-demyelinating-polyneuropathy
#20
Woo Kyung Sung, Jin Uk Jeong, Ki Tae Bang, Jong Ho Shin, Ji Hyung Yoo, Nak Min Kim, Jun Hyung Park, Joo Heon Kim
A 58-yr-old man presented with leg edema and subacute weakness of his bilateral lower extremities. Urinary and serum immunoelectrophoresis revealed the presence of lambda-type Bence Jones proteins. He was ultimately diagnosed with monoclonal gammopathy of undetermined significance (MGUS). A renal biopsy specimen showed fibrillary glomerulonephritis (FGN), which was randomly arranged as 12-20 m nonbranching fibrils in the basement membranes. Immunofluorescence studies were negative for immunoglobulin (Ig)G, IgM, IgA, C3, and kappa light chains in the capillary walls and mesangial areas...
June 2015: Kidney Research and Clinical Practice
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