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Waldenstrom macroglobulinemia

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https://www.readbyqxmd.com/read/28937595/the-diagnostic-prognostic-and-therapeutic-utility-of-molecular-testing-in-a-patient-with-waldenstrom-s-macroglobulinemia
#1
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
#2
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
#3
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
#4
REVIEW
K Growková, E Kryukova, Z Kufová, J Filipová, T Ševčíková, L Říhová, M Kaščák, F Kryukov, R 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
#5
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
#6
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
#7
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
#8
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
https://www.readbyqxmd.com/read/28801984/low-dose-bortezomib-and-dexamethasone-as-primary-therapy-in-elderly-patients-with-waldenstr%C3%B3-m-macroglobulinemia
#9
Ya-Ping Zhang, Xi Yang, Zeng-Hua Lin, Xin-Feng Wang, Xin Cao, Xue-Fen You, Hong-Ming Huang, Wen-Yu Shi, Hong Liu
OBJECTIVE: This retrospective study was designed to determine the efficacy and safety of low-dose bortezomib and dexamethasone (lBD) in elderly Chinese patients with Waldenstrӧm macroglobulinemia (WM). METHODS: Ten WM patients aged over 60 years received first-line treatment with lBD. RESULTS: The median age was 70 years (range, 61-77 years). The overall response rate was 80%, including 1 patient who achieved a complete response, 1 patient with very good partial response, and 6 patients with a partial response...
August 12, 2017: European Journal of Haematology
https://www.readbyqxmd.com/read/28798070/cxcl13-levels-are-elevated-in-patients-with-waldenstrom-s-macroglobulinemia-and-are-predictive-of-major-response-to-ibrutinib
#10
Josephine M Vos, Nickolas Tsakmaklis, Christopher J Patterson, Kirsten Meid, Jorge J Castillo, Philip Brodsky, Tomas Ganz, Steven T Pals, Marie José Kersten, Lian Xu, Guang Yang, Steven P Treon, Zachary R Hunter
No abstract text is available yet for this article.
August 10, 2017: Haematologica
https://www.readbyqxmd.com/read/28781833/edta-induced-pseudothrombocytosis-and-citrate-induced-platelet-agglutination-in-a-patient-with-waldenstrom-macroglobulinemia
#11
Pia Bükmann Larsen, Jonas Vikeså, Lennart Friis-Hansen
Hematology analyzers sometimes generate spurious results. A patient had EDTA-induced pseudothrombocytosis and platelet agglutination in citrate blood samples. This case verifies that addition of 1% paraformaldehyde to the citrate tubes can prevent platelet clumping. Further, it illustrates the advantages of having access to more than one platelet count method.
August 2017: Clinical Case Reports
https://www.readbyqxmd.com/read/28775119/incidence-and-management-of-toxicity-associated-with-ibrutinib-and-idelalisib-a-practical-approach
#12
Iris de Weerdt, Suzanne M Koopmans, Arnon P Kater, Michel van Gelder
The use of novel B cell receptor signaling inhibitors results in high response rates and long progression-free survival in patients with indolent B cell malignancies, such as chronic lymphocytic leukemia, follicular lymphoma, mantle cell lymphoma and Waldenstroms macroglobulinemia. Ibrutinib, the first-in-class inhibitor or Bruton's tyrosine kinase, and idelalisib, the first-in-class inhibitor of phosphatidylinositol 3-kinase δ have recently been approved for several indolent B cell malignancies. These drugs especially foresee in previously unmet needs for patients with relapsed or refractory disease, high-risk cytogenetic or molecular abnormalities or with comorbidities...
August 3, 2017: Haematologica
https://www.readbyqxmd.com/read/28771100/rare-transformation-to-double-hit-lymphoma-in-waldenstrom-s-macroglobulinemia
#13
Onyemaechi N Okolo, Ariel C Johnson, Seongseok Yun, Stacy J Arnold, Faiz Anwer
Waldenström macroglobulinemia (WM) is a lymphoproliferative lymphoma that is characterized by monoclonal immunoglobulin M (IgM) protein and bone marrow infiltration. Its incidence is rare and rarer still is its ability to transform to a B-cell lymphoma, particularly the aggressive diffuse large B-cell lymphoma, which bodes a poor prognosis. When transformation includes mutations of MYC, BCL-2 and/or BCL-6, it is known as a 'double hit' or 'triple hit' lymphoma respectively. This paper presents a rare case of WM with mutations positive for MYC and BCL2, making it a case of double hit B-cell lymphoplasmacytic lymphoma with plasmatic differentiation without morphological transformation to aggressive histology like DLBCL...
August 2017: Immunotherapy
https://www.readbyqxmd.com/read/28760303/analysis-of-efficacy-and-tolerability-of-bruton-tyrosine-kinase-inhibitor-ibrutinib-in-various-b-cell-malignancies-in-the-general-community-a%C3%A2-single-center-experience
#14
Naveed Ali, Faizan Malik, Syed Imran Mustafa Jafri, Mary Naglak, Mark Sundermeyer, Peter V Pickens
BACKGROUND: Ibrutinib, an irreversible inhibitor of Bruton tyrosine kinase (BTK), is a novel drug that has shown significant efficacy and survival benefit for treatment of various B-cell malignancies. The primary objective of the present study was to investigate the efficacy of ibrutinib therapy in various B-cell malignancies in the general community. The secondary objectives included studying the adverse effects, ibrutinib-induced peripheral lymphocytosis, and effect on immunoglobulin levels...
July 2017: Clinical Lymphoma, Myeloma & Leukemia
https://www.readbyqxmd.com/read/28758262/paraneoplastic-pemphigus-seen-in-4-patients-with-hematologic-malignancies-formerly-treated-with-rituximab
#15
C Baykal, S Kılıç, R Küçükoğlu
Paraneoplastic pemphigus (PNP) is a peculiar variant of pemphigus with pathognomonic clinical, histological, and immunological features. It is typically associated with hematologic malignancies (84%), such as non-Hodgkin lymphomas (NHL) (most common), chronic lymphocytic leukemia (CLL), Castleman disease, thymoma, Waldenström's macroglobulinemia, Hodgkin lymphoma, and monoclonal gammopathy, as well as non-hematological neoplasms, such as epithelial carcinomas, mesenchymal sarcomas, and malignant melanoma. This article is protected by copyright...
July 31, 2017: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/28754818/tp53-mutation-and-its-prognostic-significance-in-waldenstrom-s-macroglobulinemia
#16
Stéphanie Poulain, Christophe Roumier, Elisabeth Bertrand, Aline Renneville, Aurelie Venet-Caillault, Emmanuelle Doye, Sandrine Geffroy, Scheherazade Sebda, Olivier Nibourel, Morgane Nudel, Charles Herbaux, Loic Renaud, Cecile Tomowiak, Stéphanie Guidez, Sabine Tricot, Catherine Roche-Lestienne, Bruno Quesnel, Claude Preudhomme, Xavier Leleu
Purpose:TP53 is a tumor suppressor gene that functions as regulator influencing cellular responses to DNA damage, and TP53 alteration are associated to pejorative outcome in most of B lymphoid disorders. Little is known regarding TP53 alteration in Waldenstrom's Macroglobulinemia (WM). <p>Experimental design: Here we have explored the incidence of TP53 alteration using sanger sequencing and ultra-deep targeted sequencing in 125 WM and 10 IgM MGUS, along with the clinical features and the associated genomic landscape using SNP array and mutational landscape in an integrative study...
July 28, 2017: Clinical Cancer Research: An Official Journal of the American Association for Cancer Research
https://www.readbyqxmd.com/read/28723285/case-244-systemic-amyloidosis-a-complication-of-waldenstr%C3%A3-m-macroglobulinemia
#17
Maja Hrabak-Paar, Marko Kralik
History A 68-year-old man was admitted to the hospital for work-up because of generalized fatigue, anorexia, chronic diarrhea, and weight loss. Laboratory work-up revealed an erythrocyte sedimentation rate of 58 mm/h (reference range, 3-23 mm/h), a hemoglobin level of 14.1 g/dL (reference range, 13.8-17.5 g/dL), a leukocyte count of 8.1 × 10(9)/L (reference range, [3.4-9.7] × 10(9)/L), a platelet count of 223 × 10(9)/L (reference range, [158-424] × 10(9)/L), an alkaline phosphatase level of 85 U/L (1.42 μkat/L) (normal level, <142 U/L [2...
August 2017: Radiology
https://www.readbyqxmd.com/read/28714377/ibrutinib-associated-tumor-lysis-syndrome-in-chronic-lymphocytic-leukemia-small-lymphocytic-lymphoma-and-mantle-cell-lymphoma-a-case-series-and-review-of-the-literature
#18
Krystal S Titus-Rains, Jamie N Brown, Julia M Hammond
Background Tumor lysis syndrome results when intracellular contents are released during cell lysis. Ibrutinib, a Bruton tyrosine kinase inhibitor, is used for the treatment of chronic lymphocytic leukemia, small lymphocytic lymphoma, Waldenström's macroglobulinemia, mantle cell lymphoma, and marginal zone lymphoma. Tumor lysis syndrome caused by ibrutinib therapy is potentially life threatening, but is rare and not often reported in clinical trials. Objective The purpose of this case series is to describe the occurrence of tumor lysis syndrome in two patients initiated on ibrutinib, and to highlight the importance of close monitoring during therapy...
January 1, 2017: Journal of Oncology Pharmacy Practice
https://www.readbyqxmd.com/read/28705308/primary-sj%C3%A3-gren-s-syndrome-with-waldenstr%C3%A3-m-s-macroglobulinemia-presenting-as-unilateral-bloody-pleural-effusion
#19
Yasuhiko Koga, Kazuhiko Uchiyama Md, Norifumi Tsukamoto Md PhD, Nobuyuki Shibusawa Md PhD, Mitsuyoshi Utsugi Md PhD, Satoru Kakizaki Md PhD, Junko Hirato Md PhD, Kunio Dobashi Md PhD, Takeshi Hisada Md PhD, Masanobu Yamada Md PhD
No abstract text is available yet for this article.
July 2017: Respiratory Investigation
https://www.readbyqxmd.com/read/28687991/retrospective-analysis-of-prognostic-factors-for-waldenstr%C3%B3-m-macroglobulinemia-a-multicenter-cooperative-study-in-japan
#20
Akio Saito, Atsushi Isoda, Masaru Kojima, Akihiko Yokohama, Yutaka Tsukune, Makoto Sasaki, Shigeki Ito, Akihiro Ohtsu, Michiaki Koike, Kayoko Murayama, Keiichi Moriya, Hideto Tamura, Morio Matsumoto, Hirotaka Nakahashi, Sakae Tanosaki, Tohru Sakura, Toshihide Kawamura, Tomomi Miyanaga, Naoya Nakamura, Hirokazu Murakami, Hiroshi Handa, Norifumi Tsukamoto
Although population-based cancer registries have reported lower incidence of Waldenstrӧm macroglobulinemia (WM) in East Asia than in Western countries, previous retrospective analyses have found the clinical features of WM to be similar in these two populations. To clarify the characteristics of Japanese WM patients, we retrospectively analyzed clinical and laboratory characteristics, treatments, outcomes, and prognostic factors in 93 patients with WM. Based on the Second International Workshop on WM (IWWM-2) criteria, symptomatic WM was found in 73 (78...
July 7, 2017: International Journal of Hematology
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