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https://www.readbyqxmd.com/read/29477943/the-use-of-emergency-apheresis-in-the-management-of-plasma-cell-disorders
#1
REVIEW
Sevgi Kalayoglu-Besisik
Hyperviscosity syndrome (HVS) develops most commonly in Waldenström's macroglobulinemia (WM) and multiple myeloma (MM). Plasmapheresis is the immediate therapy and very effective at relieving symptoms by removing paraprotein. The most commonly used replacement fluid is 4%-5% human albumin in physiologic saline. FFP may be used in patients with coagulation abnormalities. Plasmapheresis should be continued until acute symptoms abate. Hyperviscosity impairs the circulation in the retina and causes hemorrhages around the small retinal vessels...
February 2018: Transfusion and Apheresis Science
https://www.readbyqxmd.com/read/29353304/unraveling-the-heterogeneity-of-igm-monoclonal-gammopathies-a-gene-mutational-and-gene-expression-study
#2
Cristina Jiménez, María Isabel Prieto-Conde, María García-Álvarez, Miguel Alcoceba, Fernando Escalante, María Del Carmen Chillón, Alfonso García de Coca, Ana Balanzategui, Alberto Cantalapiedra, Carlos Aguilar, Rocío Corral, Tomás González-López, Luis A Marín, Abelardo Bárez, Noemí Puig, Aránzazu García-Mateo, Norma C Gutiérrez, María Eugenia Sarasquete, Marcos González, Ramón García-Sanz
Immunoglobulin M (IgM) monoclonal gammopathies show considerable variability, involving three different stages of presentation: IgM monoclonal gammopathy of undetermined significance (IgM-MGUS), asymptomatic Waldenström's macroglobulinemia (AWM), and symptomatic WM (SWM). Despite recent findings about the genomic and transcriptomic characteristics of such disorders, we know little about the causes of this clinical heterogeneity or the mechanisms involved in the progression from indolent to symptomatic forms...
March 2018: Annals of Hematology
https://www.readbyqxmd.com/read/29342381/long-term-follow-up-of-monoclonal-gammopathy-of-undetermined-significance
#3
Robert A Kyle, Dirk R Larson, Terry M Therneau, Angela Dispenzieri, Shaji Kumar, James R Cerhan, S Vincent Rajkumar
BACKGROUND: Monoclonal gammopathy of undetermined significance (MGUS) occurs in approximately 3% of persons 50 years of age or older. METHODS: We studied 1384 patients who were residing in southeastern Minnesota and in whom MGUS was diagnosed at the Mayo Clinic in the period from 1960 through 1994; the median follow-up was 34.1 years (range, 0.0 to 43.6). The primary end point was progression to multiple myeloma or another plasma-cell or lymphoid disorder. RESULTS: During 14,130 person-years of follow-up, MGUS progressed in 147 patients (11%), a rate that was 6...
January 18, 2018: New England Journal of Medicine
https://www.readbyqxmd.com/read/29326807/heavy-chain-diseases-and-myeloma-associated-fanconi-syndrome-an-update
#4
REVIEW
Roberto Ria, Franco Dammacco, Angelo Vacca
The heavy chain diseases (HCDs) are rare B-cell malignancies characterized by the production of a monoclonal immunoglobulin heavy chain without an associated light chain. There are three types of HCD, defined by the class of immunoglobulin heavy chain produced: IgA (α-HCD), IgG (γ-HCD), and IgM (μ-HCD). Alpha-HCD is the most common and usually occurs as intestinal malabsorption in a young adult from a country of the Mediterranean area. Gamma- and μ-HCDs are rarer and associated with a B-cell non-Hodgkin lymphoma that produces an abnormal Ig heavy chain...
2018: Mediterranean Journal of Hematology and Infectious Diseases
https://www.readbyqxmd.com/read/29169431/monoclonal-igm-gammopathy-and-waldenstr%C3%A3-m-s-macroglobulinemia
#5
Alexander Grunenberg, Christian Buske
BACKGROUND: 3.2-3.5% of persons over age 50 have a monoclonal gammopathy. Monoclonal gammopathies have many causes, including cancer. 10-20% of monoclonal gammopathies are of isotype IgM. A systematic approach to the differential diagnosis of IgM gammopathies is essential because of the different therapeutic implications of the various underlying conditions. METHODS: This review is based on pertinent publications retrieved by a selective search in PubMed and current guidelines from Germany and abroad...
November 3, 2017: Deutsches Ärzteblatt International
https://www.readbyqxmd.com/read/29034435/advances-in-the-treatment-of-paraproteinemic-neuropathy
#6
REVIEW
Eduardo Nobile-Orazio, Mariangela Bianco, Andrea Nozza
Purpose of review Several advances have been made on the pathogenesis and therapy of neuropathies associated with paraproteinemia (monoclonal gammopathy). It is important for the neurologist to understand the pathogenetic relevance of this association especially when the hematological disease does not require per se any therapy. Recent findings Treatment of the neuropathy in patients with malignant paraproteinemia is mainly addressed by the hematologist while the neurologist is mainly involved in the initial diagnosis and in deciding whether the neuropathy is caused by the disease or by the chemotherapy used for the disease...
October 16, 2017: Current Treatment Options in Neurology
https://www.readbyqxmd.com/read/29027517/-unexpected-symptoms-of-monoclonal-gammopathy
#7
P M Smit, A C Abrahams, R G E Schutgens, N C Notermans, M C Minnema
Monoclonal gammopathy of undetermined significance (MGUS) is a common haematological disorder characterized by the presence of a monoclonal protein (M-protein). MGUS is considered an asymptomatic 'innocent' pre-malignant precursor condition of - mostly - multiple myeloma, without indication for treatment. We present three cases illustrating that MGUS can lead to serious problems. The first patient, a 51-year-old female, presented with polyneuropathy due to anti-MAG antibodies related to IgM MGUS. The second patient, a 37-year-old female, presented with proteinuria due to immunotactoid glomerulopathy caused by renal monoclonal IgG deposition associated with MGUS...
2017: Nederlands Tijdschrift Voor Geneeskunde
https://www.readbyqxmd.com/read/29021514/characteristics-and-prognosis-of-patients-with-immunoglobulin-m-monoclonal-gammopathy
#8
Hiroaki Tanaka, Yukie Sakuma, Hideki Ikeda, Ryo Shimizu, Yasumasa Sugita, Rie Iwai
Many patients with immunoglobulin M (IgM) monoclonal gammopathy remain asymptomatic and, consequently, untreated; however, few studies have evaluated the clinical course and prognosis of these patients. Using the screening procedures at our hospital, 74 patients with IgM monoclonal gammopathy were selected. We excluded 11 patients in whom the treatment for lymphoid neoplasms had been initiated at the time of IgM monoclonal protein detection. The remaining 63 patients were considered to be the patient population with IgM MGUS and asymptomatic WM, and were analyzed...
2017: Journal of Clinical and Experimental Hematopathology: JCEH
https://www.readbyqxmd.com/read/28903575/diagnostic-tools-of-waldenstr%C3%A3-ms-macroglobulinemia-best-possibilities-for-non-invasive-and-long-term-disease-monitoring
#9
K Growkova, Z Kufová, T Sevcikova, J Filipová, M Kascak, T Jelínek, S Grosicki, A Barchnicka, Ľ Roziaková, M Mistrík, M Simicek, R Hájek
Waldenströms macroglobulinemia (WM) is a B-cell malignancy characterized by high level of monoclonal immunoglobulin M (IgM) paraprotein in blood serum and associated with the bone marrow infiltration by malignant cells with lymphoplasmacytic differentiation. WM remains incurable advances in therapy. Most of WM cases are associated with a somatic point mutation L265P in MYD88. Significantly higher risk of progression from the IgM monoclonal gammopathy of undetermined significance (IgM MGUS) to WM for patients with mutated MYD88 gene suggests that this mutation is an early oncogenic event and plays a central role in development of malignant clones...
2017: Klinická Onkologie: Casopis Ceské a Slovenské Onkologické Spolecnosti
https://www.readbyqxmd.com/read/28754818/-tp53-mutation-and-its-prognostic-significance-in-waldenstrom-s-macroglobulinemia
#10
Stéphanie Poulain, Christophe Roumier, Elisabeth Bertrand, Aline Renneville, Aurélie Caillault-Venet, Emmanuelle Doye, Sandrine Geffroy, Sheherazade Sebda, Olivier Nibourel, Morgane Nudel, Charles Herbaux, Loic Renaud, Cécile Tomowiak, Stéphanie Guidez, Sabine Tricot, Catherine Roche-Lestienne, Bruno Quesnel, Claude Preudhomme, Xavier Leleu
Purpose: TP53 is a tumor-suppressor gene that functions as a regulator influencing cellular responses to DNA damage, and TP53 alterations are associated with pejorative outcome in most B-lymphoid disorders. Little is known regarding TP53 alteration in Waldenstrom's macroglobulinemia (WM). Experimental Design: Here, we have explored the incidence of TP53 alteration using Sanger sequencing and ultradeep-targeted sequencing in 125 WM and 10 immunoglobulin M (IgM) monoclonal gammopathy of undetermined significance (MGUS), along with the clinical features and the associated genomic landscape using single-nucleotide polymorphism array and mutational landscape in an integrative study...
October 15, 2017: Clinical Cancer Research: An Official Journal of the American Association for Cancer Research
https://www.readbyqxmd.com/read/28602036/circulating-adiponectin-levels-differ-between-patients-with-multiple-myeloma-and-its-precursor-disease
#11
Jonathan N Hofmann, Sham Mailankody, Neha Korde, Ye Wang, Nishant Tageja, Rene Costello, Adriana Zingone, Malin Hultcrantz, Michael N Pollak, Mark P Purdue, Ola Landgren
OBJECTIVE: An increased risk of multiple myeloma (MM) has been observed among individuals with low prediagnostic circulating levels of adiponectin, a metabolic hormone that is typically underexpressed among those with overweight or obesity. To assess whether adiponectin may influence myeloma development or progression to frank MM, circulating adiponectin levels were compared across patients with different stages of MM and its precursor, monoclonal gammopathy of undetermined significance (MGUS)...
August 2017: Obesity
https://www.readbyqxmd.com/read/28512563/acquired-von-willebrand-syndrome-associated-to-secondary-igm-mgus-emerging-after-autologous-stem-cell-transplantation-for-al-amyloidosis
#12
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
#13
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...
February 2018: Hematological Oncology
https://www.readbyqxmd.com/read/28284031/immunoparesis-in-igm-gammopathies-as-a-useful-biomarker-to-predict-disease-progression
#14
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...
August 28, 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
#15
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...
June 2017: Annals of Hematology
https://www.readbyqxmd.com/read/27974705/are-neurological-complications-of-monoclonal-gammopathy-of-undetermined-significance-underestimated
#16
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
#17
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
#18
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
#19
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
#20
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
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