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Waldenstrom macroglobulinemia

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https://www.readbyqxmd.com/read/28801984/low-dose-bortezomib-and-dexamethasone-as-primary-therapy-in-elderly-patients-with-waldenstr%C3%B3-m-macroglobulinemia
#1
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
#2
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
#3
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
#4
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
#5
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 3, 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
#6
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
#7
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
#8
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
#9
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
#10
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
#11
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
#12
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
https://www.readbyqxmd.com/read/28664939/clinical-utility-of-recently-identified-diagnostic-prognostic-and-predictive-molecular-biomarkers-in-mature-b-cell-neoplasms
#13
REVIEW
Arantza Onaindia, L Jeffrey Medeiros, Keyur P Patel
Genomic profiling studies have provided new insights into the pathogenesis of mature B-cell neoplasms and have identified markers with prognostic impact. Recurrent mutations in tumor-suppressor genes (TP53, BIRC3, ATM), and common signaling pathways, such as the B-cell receptor (CD79A, CD79B, CARD11, TCF3, ID3), Toll-like receptor (MYD88), NOTCH (NOTCH1/2), nuclear factor-κB, and mitogen activated kinase signaling, have been identified in B-cell neoplasms. Chronic lymphocytic leukemia/small lymphocytic lymphoma, diffuse large B-cell lymphoma, follicular lymphoma, mantle cell lymphoma, Burkitt lymphoma, Waldenström macroglobulinemia, hairy cell leukemia, and marginal zone lymphomas of splenic, nodal, and extranodal types represent examples of B-cell neoplasms in which novel molecular biomarkers have been discovered in recent years...
June 30, 2017: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
https://www.readbyqxmd.com/read/28655092/-evaluation-of-clinical-characteristics-myd88-l265p-mutation-cxcr4-whim-mutation-and-prognosis-in-waldenstr%C3%A3-m-macroglobulinemia-a-single-center-retrospective-study-of-93-patients
#14
X X Cao, Q Meng, H Cai, Y Y Mao, M H Duan, T N Zhu, W Zhang, B Han, J L Zhuang, H C Cai, M Chen, J Feng, X Han, Y Zhang, C Yang, L Zhang, D B Zhou, J Li
Objective: To evaluate the clinical characteristics, MYD88(L265P) mutation, CXCR4(W)HIM mutation and prognosis in patients with Waldenström macroglobulinemia (WM). Methods: The clinical characteristics, International Prognostic Scoring System for symptomatic WM (WPSS) , and overall survival (OS) were retrospectively assayed in 93 patients with newly diagnosed WM at Peking Union Medical College Hospital during January 2000 to August 2016. The MYD88(L265P) mutation and CXCR4(W)HIM mutation were tested among 34 patients...
June 14, 2017: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
https://www.readbyqxmd.com/read/28627827/a-case-of-waldenstrom-macroglobulinemia-with-temporary-appearance-of-7s-igm-half-molecule
#15
Mayumi Imoto, Koji Yoshida, Yasuhiro Maeda, Ken-Ichi Nakae, Masatoshi Kudo, Ikunosuke Sakurabayashi, Toshiyuki Yamada, Toshinori Kamisako
BACKGROUND: We encountered a rare case of Waldenstrom macroglobulinemia with temporary appearance of 7S IgM half molecule and with monoclonal proteins binding to agarose gel. METHODS: The patient's serum and urine were analyzed using sodium dodecyl sulfate-polyacrylamide gel electrophoresis and immunoblotting. The N-terminal amino acid sequences of the IgM with abnormal mass (68 kDa) were determined and compared with those of known immunoglobulin. RESULTS: The 68 kDa IgM consisted of a defective μ chain (36 kDa) and an intact κ chain...
May 1, 2017: Clinical Laboratory
https://www.readbyqxmd.com/read/28617062/novel-approaches-to-targeting-myd88-in-waldenstr%C3%A3-m-macroglobulinemia
#16
Jorge J Castillo, Zachary R Hunter, Guang Yang, Steven P Treon
Waldenström macroglobulinemia (WM) is an incurable lymphoma characterized by the accumulation of IgM-producing lymphoplasmacytic cells in the bone marrow and other organs. Although WM patients can experience prolonged remissions, the disease invariably recurs advocating for the need of novel treatments in order to achieve higher response and survival rates. The discovery of a recurrent mutation in the MYD88 gene and an increased understanding behind the biology of MYD88 signaling have provided the opportunity to developing novel agents targeting the MYD88 pathway...
August 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28592763/malignant-lymphoma-pathophysiology-and-current-therapy
#17
Koji Izutsu
Recent advances in the understanding of molecular pathogenesis of lymphoma have enabled us to clearly define disease entity by means of a disease-specific gene mutation and to select candidates for novel targeted therapy. In this review three different clinically relevant topics related to the molecular pathogenesis of lymphoma were covered. In the 2016 revision of the World Health Organization classification of lymphoid malignancies, firstly, disease-specific mutations such as MYD88 L265P in Waldenström macroglobulinemia and BRAF V600E in hairy cell leukemia were incorporated into diagnostic tests, and secondly, the determination of cell-of-origin in diffuse large B-cell lymphoma (DLBCL) was strongly recommended in diagnosis...
2017: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/28592170/current-options-to-manage-waldenstr%C3%A3-m-s-macroglobulinemia
#18
Giulia Benevolo, Maura Nicolosi, Elisa Santambrogio, Umberto Vitolo
Waldenström's macroglobulinemia (WM) is a rare, incurable B-cell lymphoma, with a median survival of 5-10 years in symptomatic patients. There is no consensus on the standard of care and several agents are currently used in these patients. Areas covered: In this article, we will review the use of standard therapies and new drugs investigated such as monoclonal antibodies, proteasome inhibitors, immunomodulatory agents, Bruton's tyrosine kinase inhibitors and novel agents in early-stage development. Expert commentary: RCD (Rituximab/Cyclophosphamide/Dexamethasone) is an effective and safe treatment in first line in WM...
June 19, 2017: Expert Review of Hematology
https://www.readbyqxmd.com/read/28588865/diffuse-large-b-cell-lymphoma-mimicking-cardiac-amyloidosis
#19
Lawrence Lau, Viktoriya Mozolevska, Iain D C Kirkpatrick, Davinder S Jassal, Roopesh Kansara
Our case highlights that cardiac lymphoma may mimic amyloid infiltration of the myocardium on cardiac magnetic resonance (CMR), and is a particularly challenging diagnosis in the setting of transformed Waldenström's macroglobulinemia. Heightened suspicion and early diagnosis of cardiac lymphoma is paramount as chemotherapy has been demonstrated to portent an increased survival rate.
June 2017: Clinical Case Reports
https://www.readbyqxmd.com/read/28583038/carfilzomib-as-salvage-therapy-in-waldenstrom-macroglobulinemia-a-case-series
#20
David H Vesole, Joshua Richter, Noa Biran, Laura McBride, Palka Anand, Mei Huang, Anita-Zahlten Kumeli, Zandra Klippel, Karim Iskander, David S Siegel
No abstract text is available yet for this article.
June 5, 2017: Leukemia & Lymphoma
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