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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/28915858/risk-of-obstetric-anal-sphincter-injury-increases-with-maternal-age-irrespective-of-parity-a-population-based-register-study
#3
Ulla Waldenström, Cecilia Ekéus
BACKGROUND: Obstetric anal sphincter injury (OASI) is a rare but serious outcome of vaginal birth. Based on concerns about the increasing number of women who commence childbearing later than previous generation, this study aimed at investigating age-related risk of OASI in women of different parity. METHODS: A population-based register study including 959,559 live singleton vaginal births recorded in the Swedish Medical Birth Register 1999 to 2011. In each parity group risks of OASI at age 25-29 years, 30-34 years, and ≥35 years compared with age < 25 years were investigated by logistic regression analyses, adjusted for year of birth, education, region of birth, smoking, Body Mass Index, infant birthweight and fetal presentation; and in parous women, history of OASI and cesarean section...
September 15, 2017: BMC Pregnancy and Childbirth
https://www.readbyqxmd.com/read/28903575/diagnostic-tools-of-waldenstr%C3%A3-ms-macroglobulinemia-best-possibilities-for-non-invasive-and-long-term-disease-monitoring
#4
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/28903570/czech-registry-of-monoclonal-gammopathies-technical-solution-data-collection-and-visualisation
#5
L Brozova, D Schwarz, I Snabl, J Kalina, B Pavlickova, M Komenda, J Jarkovský, P Němec, D Horinek, Z Stefanikova, L Pour, R Hájek, V Maisnar
BACKGROUND: The Registry of Monoclonal Gammopathies (RMG) was established by the Czech Myeloma Group in 2007. RMG is a registry designed for the collection of clinical data concerning diagnosis, treatment, treatment results and survival of patients with monoclonal gammopathies. Data on patients with monoclonal gammopathy of undetermined significance (MGUS), Waldenström macroglobulinaemia (WM), multiple myeloma (MM) or primary AL ("amyloid light-chain") amyloidosis are collected in the registry...
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
#6
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
#7
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
#8
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
#9
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/28869191/co-infection-with-babesia-divergens-and-anaplasma-phagocytophilum-in-cattle-bos-taurus-sweden
#10
Martin O Andersson, Bronislava Víchová, Conny Tolf, Sandra Krzyzanowska, Jonas Waldenström, Maria E Karlsson
Babesiosis is a severe disease in cattle worldwide. In Europe, the main causative agent of bovine babesiosis is Babesia divergens. In some areas, this species is reported to have declined or even disappeared, and its etiological role overtaken by other piroplasmid species. Moreover, co-infection with other tick-transmitted pathogens can be expected to complicate diagnosis in cattle. Hence, molecular identification of the causative agent of babesiosis should be a priority. Therefore, samples from 71 domestic cattle, 39 with clinical signs of babesiosis and 32 without, from southern Sweden were screened for Babesia spp...
August 24, 2017: Ticks and Tick-borne Diseases
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
#11
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
#12
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
#13
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
#14
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
#15
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
#16
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/28770576/transformed-waldenstr%C3%A3-m-macroglobulinaemia-clinical-presentation-and-outcome-a-multi-institutional-retrospective-study-of-77-cases-from-the-french-innovative-leukemia-organization-filo
#17
Eric Durot, Cécile Tomowiak, Anne-Sophie Michallet, Jehan Dupuis, Bénédicte Hivert, Stéphane Leprêtre, Elise Toussaint, Sophie Godet, Fatiha Merabet, Eric Van Den Neste, Sarah Ivanoff, Xavier Roussel, Jean-Marc Zini, Caroline Regny, Richard Lemal, Laurent Sutton, Aurore Perrot, Katell Le Dû, Lukshe Kanagaratnam, Pierre Morel, Véronique Leblond, Alain Delmer
Histological transformation (HT) to diffuse large B-cell lymphoma (DLBCL) is a rare and poorly reported complication of Waldenström macroglobulinaemia (WM). We performed a retrospective study of 77 WM patients with biopsy-proven transformation to DLBCL. The median time from WM diagnosis to HT was 4·6 years and 16 patients (21%) had never been treated for WM. At HT, extranodal sites were observed in 91% of patients with a rather high incidence of central nervous system, cutaneous or testicular involvement...
August 2, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28765059/bing-neel-syndrome-bilateral-cavernous-sinus-lymphoma-causing-visual-failure
#18
Chengde Pham, James D Griffiths, Anthony Kam, Martin K Hunn
We report the case of a 68-year-old male with right eye vision loss secondary to a compressive optic neuropathy from Waldenstrom macroglobulinaemia relapse in both cavernous sinuses. Central nervous system involvement is extremely uncommon in lymphoplasmacytic lymphoma. Known as Bing-Neel syndrome, this has not been previously reported to present simultaneously in bilateral cavernous sinuses. We discuss the pathophysiology, diagnostic and neuroradiological features of Bing-Neel syndrome. In this case, there was marked clinical and radiological response to chemotherapy...
July 29, 2017: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
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
#19
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
#20
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
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