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Waldenstrom macroglobulinemia

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https://www.readbyqxmd.com/read/29118506/localized-retinal-degeneration-secondary-to-waldenstr%C3%A3-m-s-macroglobulinemia
#1
Dhanashree Ratra, Vineet Ratra, Mansi Kishnani
A 52-year-old man, treated for Waldenström's macroglobulinemia (WM), continued to experience decreased vision even after 24 months. He was evaluated using multimodal imaging and electroretinography. The retina did not show any hyperviscosity changes but revealed a yellow lesion at macula with atrophic changes causing a pattern on fluorescein angiography similar to a leopard's skin. Optical coherence tomographic imaging revealed uniformly reflective material deposited in the outer retina with degeneration of outer retinal layers...
September 2017: Oman Journal of Ophthalmology
https://www.readbyqxmd.com/read/29113857/a-rare-neurological-complication-of-waldenstrom-s-macroglobulinemia
#2
Gabriel Torrealba-Acosta, Rajan Gadhia, Thabele Leslie-Mazwi
Bilateral and simultaneous facial nerve palsy (FNP) is a rare clinical condition occurring in 0.3-2.0% of facial palsy cases and is typically a manifestation of an underlying systemic disease. We here describe a case of a 67-year-old Hispanic man with a known history of Waldenstrom's Macroglobulinemia (WM) who presented to the clinic with a sub-acute onset of bilateral facial weakness. No alternate etiology for the facial weakness was identified after a thorough diagnostic approach. WM is a rare hematological condition due to low-grade B cell lymphoma, where lymphoplasmacytoid cells infiltrate different tissues and secrete monoclonal IgM...
November 4, 2017: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
https://www.readbyqxmd.com/read/29081197/-clinical-features-and-prognosis-of-17-patients-with-primary-cold-agglutinin-disease
#3
Y Y Wu, X X Cao, H Cai, L Zhang, D B Zhou, J Li
Objective: To explore the clinical characteristics, treatment and prognosis in 17 patients with primary cold agglutinin disease (CAD) . Methods: Clinical data, treatment and survival status of 17 patients diagnosed with primary cold agglutinin disease in Peking Union Medical College Hospital during April 2007 to October 2016 were retrospectively analyzed. The MYD88(L265P) mutation was tested in 4 patients. Results: The median age of 17 patients was 67 years (range, 51-86 years) , and male- to female ratio was 1...
September 14, 2017: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
https://www.readbyqxmd.com/read/29080258/impact-of-myd88-mutation-status-in-waldenstr%C3%A3-m-macroglobulinemia
#4
Jithma P Abeykoon, Jonas Paludo, Rebecca L King, Stephen M Ansell, Morie A Gertz, Betsy R LaPlant, Alese E Halvorson, Wilson I Gonsalves, David Dingli, Hong Fang, S Vincent Rajkumar, Martha Q Lacy, Rong He, Taxiarchis Kourelis, Craig B Reeder, Anne J Novak, Ellen McPhail, David Viswanatha, Thomas E Witzig, Ronald S Go, Thomas Habermann, Francis K Buadi, Angela Dispenzieri, Nelson Leung, Yi Lin, Carrie Thompson, Suzanne Hayman, Robert A Kyle, Shaji Kumar, Prashant Kapoor
Waldenström macroglobulinemia (WM) is an immunoglobulin M-associated lymphoma, with majority of cases demonstrating MYD88 locus alteration, most commonly, MYD88(L265P) . Due to low prevalence of the wild-type (WT) MYD88 genotype in WM, clinically-relevant data in this patient-population are sparse, with one study showing nearly a 10-fold increased risk of mortality in this subgroup compared to patients with MYD88(L265P) mutation. We studied a large cohort of patients with MYD88(L265P) and MYD88(WT) WM, evaluated at Mayo Clinic, Rochester, between 1995 and 2016 to specifically assess the impact of these genotypes on clinical course...
October 28, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/29066491/igm-myeloma-with-plasma-cell-leukemia-case-report-and-literature-review
#5
Saurabh Chhabra, Sandeep Jain, Amanda Fowler, Valeriy Sedov, Amarendra K Neppalli, Cynthia A Schandl, John Lazarchick
IgM multiple myeloma (MM) is a rare entity representing approximately 0.5% of all MM. It should be distinguished from malignant neoplasms of B cells with plasmacytic differentiation such as Waldenstrom macroglobulinemia (WM) and marginal zone lymphoma with plasmacytic differentiation. Plasma cell leukemia (PCL) is a rare and aggressive variant of MM characterized by the presence of circulating plasma cells. We present a case report of a patient who presented with IgM MM in primary PCL phase with high-risk cytogenetics...
September 2017: Annals of Clinical and Laboratory Science
https://www.readbyqxmd.com/read/29053157/current-applications-of-multiparameter-flow-cytometry-in-plasma-cell-disorders
#6
REVIEW
T Jelinek, R Bezdekova, M Zatopkova, L Burgos, M Simicek, T Sevcikova, B Paiva, R Hajek
Multiparameter flow cytometry (MFC) has become standard in the management of patients with plasma cell (PC) dyscrasias, and could be considered mandatory in specific areas of routine clinical practice. It plays a significant role during the differential diagnostic work-up because of its fast and conclusive readout of PC clonality, and simultaneously provides prognostic information in most monoclonal gammopathies. Recent advances in the treatment and outcomes of multiple myeloma led to the implementation of new response criteria, including minimal residual disease (MRD) status as one of the most relevant clinical endpoints with the potential to act as surrogate for survival...
October 20, 2017: Blood Cancer Journal
https://www.readbyqxmd.com/read/29044604/epstein-barr-virus-positive-mucocutaneous-ulcer-in-a-background-of-crohn-s-disease-and-waldenstr%C3%A3-m-macroglobulinemia-a-case-report-highlighting-diagnostic-pitfalls
#7
Larissa Sena Teixeira Mendes, James McCaul, Andrew Wotherspoon, Ayoma D Attygalle
A specialist haematopathology opinion was sought on a left palatine tonsillar excision biopsy performed for suspected carcinoma. The patient, a 72-year old male, presented with oropharyngeal dysphagia in May 2015 and was diagnosed with hypertrophic, ulcerated lichen planus on an incision biopsy of a white patch on the posterior third of the tongue. Topical steroids were commenced with clinical improvement. This article is protected by copyright. All rights reserved.
October 16, 2017: Histopathology
https://www.readbyqxmd.com/read/29034435/advances-in-the-treatment-of-paraproteinemic-neuropathy
#8
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/29025291/-waldenstr%C3%A3-m-s-macroglobulinemia-and-its-individualized-therapy-options
#9
Zsuzsanna Szemlaky, Gábor Mikala
Waldenström's macroglobulinaemia is a form of lymphoplasmocytic lymphoma that preferentially localizes to the bone marrow and causes a special syndrome characterized by monoclonal IgM hypersecretion. Recent results point to the fact that this disease has at least three different pathobiological forms with different clinical presentation. While mutations of MYD88 occur in 95-97% of the cases, there are CXCR4 mutations in 30-40%, ARID1A mutations in 17% and CD79B mutations in approximately 10% of afflicted individuals...
October 2017: Orvosi Hetilap
https://www.readbyqxmd.com/read/28989285/unintended-target-effect-of-anti-bcl-2-dnai
#10
Abdul Shukkur Ebrahim, Mustapha Kandouz, Nada Emara, Amara B Sugalski, Leonard Lipovich, Ayad M Al-Katib
INTRODUCTION: Previous research suggested that a novel compound PNT2258 inhibits B-cell lymphoma 2 (BCL-2) transcription by DNA interference (DNAi) and demonstrated its activity in preclinical xenograft models and in a pilot Phase II clinical trial in non-Hodgkin's lymphoma (NHL). While the drug downregulates BCL-2 at the promoter, mRNA, and protein levels, there is a significant homology (13-16 bases) between PNT100 and a number of promoters of genes involved in cell cycle regulation and survival...
2017: Cancer Management and Research
https://www.readbyqxmd.com/read/28983055/pattern-of-somatic-mutations-in-patients-with-waldenstr%C3%A3-m-macroglobulinemia-or-igm-monoclonal-gammopathy-of-undetermined-significance
#11
Marzia Varettoni, Silvia Zibellini, Irene Defrancesco, Virginia Valeria Ferretti, Ettore Rizzo, Luca Malcovati, Anna Gallì, Matteo Giovanni Della Porta, Emanuela Boveri, Luca Arcaini, Chiara Candido, Marco Paulli, Mario Cazzola
We analyzed MYD88 and CXCR4 mutation status of 260 patients with Waldenström macroglobulinemia or IgM monoclonal gammopathy of undetermined significance using allele-specific real time quantitative PCR and Sanger sequencing respectively. A subgroup of 119 patients was further studied with next-generation sequencing of 11 target genes (MYD88, CXCR4, ARID1-A, KMT2D, NOTCH2, TP53, PRDM1, CD79B, TRAF3, MYBBP1A, TNFAIP3). MYD88 (L265P) was found at diagnosis in 91% of patients with Waldenström macroglobulinemia and in 60% of patients with IgM monoclonal gammopathy of undetermined significance using allele-specific PCR...
October 5, 2017: Haematologica
https://www.readbyqxmd.com/read/28958786/-lymphomas-a-therapeutic-update
#12
REVIEW
A Wolfromm, R Delarue
Emergence of new molecules has considerably reshaped the management of patients in onco-hematology. Cytotoxic chemotherapy has not been altered, and CHOP remains the reference treatment for lymphomas. However, the development of targeted therapies has allowed for a broader spectrum of treatments. Immunotherapy with monoclonal antibodies entered the market with rituximab in diffuse large B-cell lymphomas, in the 1990s and it is now developing as new-generation anti-CD20 antibodies (obinotuzumab and ofatumumab)...
October 2017: La Revue de Médecine Interne
https://www.readbyqxmd.com/read/28937595/the-diagnostic-prognostic-and-therapeutic-utility-of-molecular-testing-in-a-patient-with-waldenstrom-s-macroglobulinemia
#13
Collin K Chin, Connull Leslie, Carolyn S Grove, Chris Van Vliet, Chan Yoon Cheah
The application of molecular genomics and our understanding of its clinical implications in the diagnosis, prognostication and treatment of lymphoproliferative disorders has rapidly evolved over the past few years. Of particular importance are indolent B-cell malignancies where tumour cell survival and proliferation are commonly driven by mutations involving the B-cell receptor and downstream signalling pathways. In addition, the increasing number of novel therapies and targeted agents have provided clinicians with new therapeutic options with the aim of exploiting such mutations...
September 22, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28924457/bach2-promotes-indolent-clinical-presentation-in-waldenstr%C3%A3-m-macroglobulinemia
#14
Charles Herbaux, Elisabeth Bertrand, Guillemette Marot, Christophe Roumier, Nicolas Poret, Valérie Soenen, Olivier Nibourel, Catherine Roche-Lestienne, Natacha Broucqsault, Sylvie Galiègue-Zouitina, Eileen M Boyle, Guillemette Fouquet, Aline Renneville, Sabine Tricot, Franck Morschhauser, Claude Preudhomme, Bruno Quesnel, Stephanie Poulain, Xavier Leleu
Approximately 30% of the patients who fulfil the criteria of Waldenström's macroglobulinemia (WM) are diagnosed while asymptomatic (indolent), and will not require immediate therapy. Conversely, patients with a disease-related event will be considered for therapy. The physiopathology of these 2 groups remains unclear, and the mechanisms of progression from indolent to symptomatic WM have yet to be fully understood. Seventeen patients diagnosed with WM were included in this study, 8 asymptomatic WM (A-WM) and 9 symptomatic WM (S-WM)...
August 22, 2017: Oncotarget
https://www.readbyqxmd.com/read/28903575/diagnostic-tools-of-waldenstr%C3%A3-ms-macroglobulinemia-best-possibilities-for-non-invasive-and-long-term-disease-monitoring
#15
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/28886236/waldenstr%C3%A3-m-s-macroglobulinemia-two-malignant-clones-in-a-monoclonal-disease-molecular-background-and-clinical-reflection
#16
REVIEW
Kateřina Growková, Elena Kryukova, Zuzana Kufová, Jana Filipová, Tereza Ševčíková, Lucie Říhová, Michal Kaščák, Fedor Kryukov, Roman Hájek
Waldenström's macroglobulinemia (WM) is a complex disease characterized by apparent morphological heterogeneity within the malignant clonal cells representing a continuum of small lymphocytes, plasmacytoid lymphocytes, and plasma cells. At the molecular level, the neoplastic B cell-derived clone has undergone somatic hypermutation, but not isotype switching, and retains the capability of plasmacytic differentiation. Although by classical definition, WM is formed by monoclonal expansion, long-lived clonal B lymphocytes are of heterogeneous origin...
September 8, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/28886228/the-serum-heavy-light-chain-immunoassay-a-valuable-tool-for-sensitive-paraprotein-assessment-risk-and-disease-monitoring-in-monoclonal-gammopathies
#17
Christine Greil, Gabriele Ihorst, Felix Gaiser, Ulrich Salzer, Emanuel Bisse, Efstathios Kastritis, Heinz Ludwig, Ralph Wäsch, Monika Engelhardt
OBJECTIVE: The heavy/light chain (HLC)-immunoassay quantifies light chain types of each immunoglobulin class in patients with monoclonal gammopathies. METHODS: We assessed 147 consecutive patients with different forms and stages of plasma cell dyscrasias (PCD) who received standard tests (serum and urine protein electrophoresis [SPEP, UPEP], immunofixation [IFE], serum-free light chain [SFLC]), and HLC-immunoassay. Patients with multiple myeloma (MM, n = 102), smoldering MM (SMM, n = 5), monoclonal gammopathy of undetermined significance (MGUS, n = 28), and Waldenström's macroglobulinemia (WM, n = 12) were included...
September 8, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/28883220/a-case-of-waldenstrom-macroglobulinemia-in-which-intermittent-one-day-administration-cycles-of-bendamustine-were-effective-for-alleviation-of-nausea-and-maintenance-of-remission
#18
Yasunobu Sekiguchi, Mutsumi Wakabayashi, Haruko Takizawa, Keiji Sugimoto, Shigeki Tomita, Hiroshi Izumi, Noriko Nakamura, Tomohiro Sawada, Yasunori Ohta, Norio Komatsu, Masaaki Noguchi
No abstract text is available yet for this article.
September 6, 2017: Journal of Clinical and Experimental Hematopathology: JCEH
https://www.readbyqxmd.com/read/28877839/significances-of-myd88-l265p-and-cxcr4-whim-mutations-in-waldenstrom-macroglobulinemia
#19
Qi Meng, Xin-Xin Cao, Jian Li
Waldenstrom macroglobulinemia(WM) is a lymphoplasmacytic lymphoma characterized by serum monoclonal IgM immunoglobulin.Recently,the high mutation rates of MYD88(L265P) and CXCR4(WHIM) have been documented in WM.Furthermore,MYD88(L265P) and CXCR4(WHIM) are related to the response to target drugs.This article reviews the significances of MYD88(L265P) and CXCR4(WHIM) in the diagnosis and treatment of WM.
August 20, 2017: Zhongguo Yi Xue Ke Xue Yuan Xue Bao. Acta Academiae Medicinae Sinicae
https://www.readbyqxmd.com/read/28841204/from-waldenstr%C3%A3-m-s-macroglobulinemia-to-aggressive-diffuse-large-b-cell-lymphoma-a-whole-exome-analysis-of-abnormalities-leading-to-transformation
#20
C Jiménez, S Alonso-Álvarez, M Alcoceba, G R Ordóñez, M García-Álvarez, M I Prieto-Conde, M C Chillón, A Balanzategui, R Corral, L A Marín, N C Gutiérrez, N Puig, M E Sarasquete, M González, R García-Sanz
Transformation of Waldenström's macroglobulinemia (WM) to diffuse large B-cell lymphoma (DLBCL) occurs in up to 10% of patients and is associated with an adverse outcome. Here we performed the first whole-exome sequencing study of WM patients who evolved to DLBCL and report the genetic alterations that may drive this process. Our results demonstrate that transformation depends on the frequency and specificity of acquired variants, rather than on the duration of its evolution. We did not find a common pattern of mutations at diagnosis or transformation; however, there were certain abnormalities that were present in a high proportion of clonal tumor cells and conserved during this transition, suggesting that they have a key role as early drivers...
August 25, 2017: Blood Cancer Journal
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