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Alexis Talbot - One of the best experts on this subject based on the ideXlab platform.
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Central nervous system involvement by malignant cells is a rare complication of Waldenström Macroglobulinemia, and this clinicopathological entity is referred to as the Bing-Neel syndrome. There is currently no consensus on the diagnostic criteria, t
Haematologica, 2015Co-Authors: Laure Simon, Richard Lemal, Aikaterini Fitsiori, Jehan Dupuis, Benjamin Carpentier, Laurys Boudin, Anne Corby, Lauris Gastaud, Thérèse Aurran-schleinitz, Alexis TalbotAbstract:Central nervous system involvement by malignant cells is a rare complication of Waldenström Macroglobulinemia, and this clinicopathological entity is referred to as the Bing-Neel syndrome. There is currently no consensus on the diagnostic criteria, therapeutic approaches and response evaluation for this syndrome. In this series, we retrospectively analyzed 44 French patients with Bing-Neel syndrome. Bing-Neel syndrome was the first manifestation of Waldenström Macroglobulinemia in 36% of patients. When Waldenström Macroglobulinemia was diagnosed prior to Bing-Neel syndrome, the median time interval between this diagnosis and the onset of Bing-Neel syndrome was 8.9 years. This study highlights the possibility of the occurrence of Bing-Neel syndrome without any other evidence of progression of Waldenström Macroglobulinemia. The clinical presentation was heterogeneous without any specific signs or symptoms. Biologically, the median lymphocyte count in the cerebrospinal fluid was 31/mm(3). Magnetic resonance imaging revealed abnormalities in 78% of the cases. The overall response rate after first-line treatment was 70%, and the overall survival rate after the diagnosis of Bing-Neel syndrome was 71% at 5 years. Altogether, these results suggest that Bing-Neel syndrome should be considered in the context of any unexplained neurological symptoms associated with Waldenström Macroglobulinemia. The diagnostic approach should be based on cerebrospinal fluid analysis and magnetic resonance imaging of the brain and spinal axis. It still remains difficult to establish treatment recommendations or prognostic factors in the absence of large-scale, prospective, observational studies.
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bing neel syndrome a rare complication of waldenstrom Macroglobulinemia analysis of 44 cases and review of the literature a study on behalf of the french innovative leukemia organization filo
Haematologica, 2015Co-Authors: Laurence Simon, Richard Lemal, Aikaterini Fitsiori, Jehan Dupuis, Benjamin Carpentier, Laurys Boudin, Anne Corby, Therese Aurranschleinitz, Lauris Gastaud, Alexis TalbotAbstract:Central nervous system involvement by malignant cells is a rare complication of Waldenstrom Macroglobulinemia, and this clinicopathological entity is referred to as the Bing-Neel syndrome. There is currently no consensus on the diagnostic criteria, therapeutic approaches and response evaluation for this syndrome. In this series, we retrospectively analyzed 44 French patients with Bing-Neel syndrome. Bing-Neel syndrome was the first manifestation of Waldenstrom Macroglobulinemia in 36% of patients. When Waldenstrom Macroglobulinemia was diagnosed prior to Bing-Neel syndrome, the median time interval between this diagnosis and the onset of Bing-Neel syndrome was 8.9 years. This study highlights the possibility of the occurrence of Bing-Neel syndrome without any other evidence of progression of Waldenstrom Macroglobulinemia. The clinical presentation was heterogeneous without any specific signs or symptoms. Biologically, the median lymphocyte count in the cerebrospinal fluid was 31/mm3. Magnetic resonance imaging revealed abnormalities in 78% of the cases. The overall response rate after first-line treatment was 70%, and the overall survival rate after the diagnosis of Bing-Neel syndrome was 71% at 5 years. Altogether, these results suggest that Bing-Neel syndrome should be considered in the context of any unexplained neurological symptoms associated with Waldenstrom Macroglobulinemia. The diagnostic approach should be based on cerebrospinal fluid analysis and magnetic resonance imaging of the brain and spinal axis. It still remains difficult to establish treatment recommendations or prognostic factors in the absence of large-scale, prospective, observational studies.
Morie A. Gertz - One of the best experts on this subject based on the ideXlab platform.
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Emerging therapeutic options for Waldenström Macroglobulinemia/lymphoplasmacytic lymphoma
Expert review of anticancer therapy, 2015Co-Authors: Rajshekhar Chakraborty, Stephen M. Ansell, Prashant Kapoor, Morie A. GertzAbstract:Lymphoplasmacytic lymphoma is an indolent B-cell, non-Hodgkin lymphoma (NHL), the majority of which are characterized by production of a monoclonal immunoglobulin M (IgM) protein and are known as Waldenstrom Macroglobulinemia. Identification of highly recurrent activating somatic mutation in MYD88 has improved our understanding of the pathogenesis of Waldenstrom Macroglobulinemia and has therapeutic implications. Here, we review novel therapeutic agents in Waldenstrom Macroglobulinemia/lymphoplasmacytic lymphoma, which have emerged in the past decade and discuss their comparative efficacy and safety, with emphasis on a Bruton’s tyrosine kinase (BTK) inhibitor, which has been recently approved by the US FDA, specifically for Waldenstrom Macroglobulinemia/lymphoplasmacytic lymphoma. Future research should focus on identifying targeted agents against activating mutations and long-term data for currently available novel agents should be critically evaluated, both in treatment-naive and in relapsed/refractory ...
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Waldenström Macroglobulinemia: my way
Leukemia & lymphoma, 2012Co-Authors: Morie A. GertzAbstract:AbstractWaldenstrom Macroglobulinemia is a lymphoplasmacytic lymphoma. A serum monoclonal IgM protein is required to establish this diagnosis. The clinical features patients develop include normochromic normocytic anemia, thrombocytopenia, hepatosplenomegaly, lymphadenopathy and signs of hyperviscosity. The International Staging System for Waldenstr m Macroglobulinemia divides patients prognostically based on age, hemoglobin, platelet count, IgM level, and β2 microglobulin. Some patients with Waldenstr m Macroglobulinemia have a smoldering form and can be observed without intervention. Active agents in the treatment of Waldenstr m Macroglobulinemia include rituximab, chlorambucil, cyclophosphamide, fludarabine, bortezomib, lenalidomide, bendamustine, everolimus, and alemtuzumab. The current preferred Mayo Clinic non-study treatment is rituximab, cyclophosphamide, and dexamethasone. The median survival associated with this disease is now over 10 years.
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The use of cell lines for the study of Waldenström's Macroglobulinemia.
Leukemia research, 2008Co-Authors: Morie A. Gertz, Stephen M. AnsellAbstract:In this issue of Leukemia Research, Drexler and MacLeod raise questions about the origins of cell lines used in the study of Waldenstrom's Macroglobulinemia and suggest caution in the use of these cell lines as models of this disease [Drexler HG, Macleod RAF. Malignant hematopoietic cell lines: in vitro models for the studies of Waldenstrom's Macroglobulinemia. Leuk Res 2008].
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Waldenström Macroglobulinemia: a review of therapy.
American journal of hematology, 2005Co-Authors: Morie A. GertzAbstract:Waldenstrom Macroglobulinemia is a rare monoclonal gammopathy-associated lymphoplasmacytic lymphoma. Its incidence is only 4 per million per year. This review contains the known published literature specifically on the available management tools for Waldenstrom Macroglobulinemia and is designed to assist clinicians in making management decisions for patients with this uncommon disorder.
Steven P. Treon - One of the best experts on this subject based on the ideXlab platform.
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Initial Evaluation of the Patient with Waldenström Macroglobulinemia
Hematology oncology clinics of North America, 2018Co-Authors: Jorge J. Castillo, Steven P. TreonAbstract:The initial evaluation of the patient with Waldenstrom Macroglobulinemia can be challenging. Not only is it a rare disease, but the clinical features can vary greatly from patient to patient. In this article, we aim at providing concise and practical recommendations for the initial evaluation of patients with Waldenstrom Macroglobulinemia, specifically regarding history taking, physical examination, laboratory testing, bone marrow aspiration, and biopsy evaluation and imaging studies. We then review the most common special clinical situations seen in patients with Waldenstrom Macroglobulinemia, especially anemia, hyperviscosity, cryoglobulinemia, peripheral neuropathy, extramedullary disease, Bing-Neel syndrome, and amyloidosis.
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myd88 l265p somatic mutation in waldenstrom s Macroglobulinemia
The New England Journal of Medicine, 2012Co-Authors: Steven P. Treon, Guang Yang, Yangsheng Zhou, Xia Liu, Yang Cao, Patricia Sheehy, Robert Manning, Christopher J Patterson, Christina K Tripsas, Luca ArcainiAbstract:A b s t r ac t Background Waldenstrom’s Macroglobulinemia is an incurable, IgM-secreting lymphoplasmacytic lymphoma (LPL). The underlying mutation in this disorder has not been delineated. Methods We performed whole-genome sequencing of bone marrow LPL cells in 30 patients with Waldenstrom’s Macroglobulinemia, with paired normal-tissue and tumor-tissue sequencing in 10 patients. Sanger sequencing was used to validate the findings in samples from an expanded cohort of patients with LPL, those with other B-cell disorders that have some of the same features as LPL, and healthy donors. Results Among the patients with Waldenstrom’s Macroglobulinemia, a somatic variant (T→C) in LPL cells was identified at position 38182641 at 3p22.2 in the samples from all 10 patients with paired tissue samples and in 17 of 20 samples from patients with unpaired samples. This variant predicted an amino acid change (L265P) in MYD88, a mutation that triggers IRAK-mediated NF-κB signaling. Sanger sequencing identified MYD88 L265P in tumor samples from 49 of 54 patients with Waldenstrom’s Macroglobulinemia and in 3 of 3 patients with non–IgM-secreting LPL (91% of all patients with LPL). MYD88 L265P was absent in paired normal tissue samples from patients with Waldenstrom’s Macroglobulinemia or non-IgM LPL and in B cells from healthy donors and was absent or rarely expressed in samples from patients with multiple myeloma, marginal-zone lymphoma, or IgM monoclonal gammopathy of unknown significance. Inhibition of MYD88 signaling reduced IκBα and NF-κB p65 phosphorylation, as well as NF-κB nuclear staining, in Waldenstrom’s Macroglobulinemia cells expressing MYD88 L265P. Somatic variants in ARID1A in 5 of 30 patients (17%), leading to a premature stop or frameshift, were also identified and were associated with an increased disease burden. In addition, 2 of 3 patients with Waldenstrom’s Macroglobulinemia who had wild-type MYD88 had somatic variants in MLL2. Conclusions MYD88 L265P is a commonly recurring mutation in patients with Waldenstrom’s Macroglobulinemia that can be useful in differentiating Waldenstrom’s Macroglobulinemia and non-IgM LPL from B-cell disorders that have some of the same features. (Funded by the Peter and Helen Bing Foundation and others.)
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Novel Agents in the Treatment of Waldenström's Macroglobulinemia
Clinical Lymphoma and Myeloma, 2007Co-Authors: Steven P. Treon, Aldo M. Roccaro, Xavier Leleu, Evdoxia Hatjiharissi, Anne-sophie Moreau, Zachary R. Hunter, Jacob D. Soumerai, Bryan Ciccarelli, Antonio SaccoAbstract:Abstract Waldenstrom's Macroglobulinemia is a B-cell disorder characterized by bone marrow infiltration with lymphoplasmacytic cells and demonstration an immunoglobulin M monoclonal gammopathy. Despite advances in therapy, Waldenstrom's Macroglobulinemia remains incurable. As therapeutic agents are needed for the treatment of Waldenstrom's Macroglobulinemia. In ongoing efforts, we and others have sought to exploit made in the understanding of the biology of Waldenstrom's Macroglobulinemia so as to better target therapeutics for this malignancy. Importantly, part of these efforts, we have prioritized the development of stem cell–sparing drugs because autologous stem cell transplantation remains salvage option in Waldenstrom's Macroglobulinemia. These efforts have led to the development of several novel agents for treating Waldenstrom's Macroglobulinemia, including bortezomib; monoclonal antibodies and/or blocking protein targeting CD40, CD52, or CD70, a proliferation- ligand and B-lymphocyte stimulator; the immunomodulator thalidomide as an enhancer of rituximab activity, as well as agents interfering cell factor, phosphatidylinositol 3-kinase/Akt, phosphodiesterase, cholesterol, and protein kinase C β signaling. This report provides biologic studies and clinical efforts for the development of these novel agents in the treatment of Waldenstrom's Macroglobulinemia.
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Novel agents in the treatment of Waldenström's Macroglobulinemia.
Clinical lymphoma & myeloma, 2007Co-Authors: Steven P. Treon, Xavier Leleu, Evdoxia Hatjiharissi, Anne-sophie Moreau, Zachary R. Hunter, Jacob D. Soumerai, Bryan Ciccarelli, Aldo Roccaro, Antonio SaccoAbstract:Waldenström's Macroglobulinemia is a B-cell disorder characterized by bone marrow infiltration with lymphoplasmacytic cells and demonstration of an immunoglobulin M monoclonal gammopathy. Despite advances in therapy, Waldenström's Macroglobulinemia remains incurable. As such, novel therapeutic agents are needed for the treatment of Waldenström's Macroglobulinemia. In ongoing efforts, we and others have sought to exploit advances made in the understanding of the biology of Waldenström's Macroglobulinemia so as to better target therapeutics for this malignancy. Importantly, as part of these efforts, we have prioritized the development of stem cell-sparing drugs because autologous stem cell transplantation remains a viable salvage option in Waldenström's Macroglobulinemia. These efforts have led to the development of several novel agents for treating Waldenström's Macroglobulinemia, including bortezomib; monoclonal antibodies and/or blocking protein targeting CD40, CD52, or CD70, a proliferation-inducing ligand and B-lymphocyte stimulator; the immunomodulator thalidomide as an enhancer of rituximab activity, as well as agents interfering with stem cell factor, phosphatidylinositol 3-kinase/Akt, phosphodiesterase, cholesterol, and protein kinase C beta signaling. This report provides an update on biologic studies and clinical efforts for the development of these novel agents in the treatment of Waldenström's Macroglobulinemia.
Richard Lemal - One of the best experts on this subject based on the ideXlab platform.
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Central nervous system involvement by malignant cells is a rare complication of Waldenström Macroglobulinemia, and this clinicopathological entity is referred to as the Bing-Neel syndrome. There is currently no consensus on the diagnostic criteria, t
Haematologica, 2015Co-Authors: Laure Simon, Richard Lemal, Aikaterini Fitsiori, Jehan Dupuis, Benjamin Carpentier, Laurys Boudin, Anne Corby, Lauris Gastaud, Thérèse Aurran-schleinitz, Alexis TalbotAbstract:Central nervous system involvement by malignant cells is a rare complication of Waldenström Macroglobulinemia, and this clinicopathological entity is referred to as the Bing-Neel syndrome. There is currently no consensus on the diagnostic criteria, therapeutic approaches and response evaluation for this syndrome. In this series, we retrospectively analyzed 44 French patients with Bing-Neel syndrome. Bing-Neel syndrome was the first manifestation of Waldenström Macroglobulinemia in 36% of patients. When Waldenström Macroglobulinemia was diagnosed prior to Bing-Neel syndrome, the median time interval between this diagnosis and the onset of Bing-Neel syndrome was 8.9 years. This study highlights the possibility of the occurrence of Bing-Neel syndrome without any other evidence of progression of Waldenström Macroglobulinemia. The clinical presentation was heterogeneous without any specific signs or symptoms. Biologically, the median lymphocyte count in the cerebrospinal fluid was 31/mm(3). Magnetic resonance imaging revealed abnormalities in 78% of the cases. The overall response rate after first-line treatment was 70%, and the overall survival rate after the diagnosis of Bing-Neel syndrome was 71% at 5 years. Altogether, these results suggest that Bing-Neel syndrome should be considered in the context of any unexplained neurological symptoms associated with Waldenström Macroglobulinemia. The diagnostic approach should be based on cerebrospinal fluid analysis and magnetic resonance imaging of the brain and spinal axis. It still remains difficult to establish treatment recommendations or prognostic factors in the absence of large-scale, prospective, observational studies.
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TCL1 expression patterns in Waldenström Macroglobulinemia.
Modern pathology : an official journal of the United States and Canadian Academy of Pathology Inc, 2015Co-Authors: Richard Lemal, Sandrine Bard-sorel, Laura Montrieul, Jacques-olivier Bay, Aurélie Ravinet, Albane Ledoux-pilon, Nicolas Cagnard, Sébastien Bailly, Pierre Morel, Frédéric CharlotteAbstract:The oncogenic role of TCL1 in chronic lymphocytic leukemia is well established in transgenic mice. TCL1 expression in other B-cell malignancies has been also described: post-germinal center-derived malignancies, such as multiple myeloma, classically do not express TCL1. Waldenstrom Macroglobulinemia is a post-germinal center malignancy that is known to be similar to chronic lymphocytic leukemia in terms of its gene expression profile. TCL1 expression has not been so far assessed in Waldenstrom Macroglobulinemia. Transcriptomic explorations show that TCL1A expression is linked to signaling pathways and biological functions that are known to be involved in Waldenstrom Macroglobulinemia as well as to gene signatures of interest in B-cell malignancies. We investigated TCL1 expression at the protein level in the bone marrow of a series of 59 patients with Waldenstrom Macroglobulinemia: 76% of patients expressed TCL1, which appeared to be associated with a pejorative prognostic impact. TCL1 could have an oncogenic role in Waldenstrom Macroglobulinemia, and deserves further exploration.
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bing neel syndrome a rare complication of waldenstrom Macroglobulinemia analysis of 44 cases and review of the literature a study on behalf of the french innovative leukemia organization filo
Haematologica, 2015Co-Authors: Laurence Simon, Richard Lemal, Aikaterini Fitsiori, Jehan Dupuis, Benjamin Carpentier, Laurys Boudin, Anne Corby, Therese Aurranschleinitz, Lauris Gastaud, Alexis TalbotAbstract:Central nervous system involvement by malignant cells is a rare complication of Waldenstrom Macroglobulinemia, and this clinicopathological entity is referred to as the Bing-Neel syndrome. There is currently no consensus on the diagnostic criteria, therapeutic approaches and response evaluation for this syndrome. In this series, we retrospectively analyzed 44 French patients with Bing-Neel syndrome. Bing-Neel syndrome was the first manifestation of Waldenstrom Macroglobulinemia in 36% of patients. When Waldenstrom Macroglobulinemia was diagnosed prior to Bing-Neel syndrome, the median time interval between this diagnosis and the onset of Bing-Neel syndrome was 8.9 years. This study highlights the possibility of the occurrence of Bing-Neel syndrome without any other evidence of progression of Waldenstrom Macroglobulinemia. The clinical presentation was heterogeneous without any specific signs or symptoms. Biologically, the median lymphocyte count in the cerebrospinal fluid was 31/mm3. Magnetic resonance imaging revealed abnormalities in 78% of the cases. The overall response rate after first-line treatment was 70%, and the overall survival rate after the diagnosis of Bing-Neel syndrome was 71% at 5 years. Altogether, these results suggest that Bing-Neel syndrome should be considered in the context of any unexplained neurological symptoms associated with Waldenstrom Macroglobulinemia. The diagnostic approach should be based on cerebrospinal fluid analysis and magnetic resonance imaging of the brain and spinal axis. It still remains difficult to establish treatment recommendations or prognostic factors in the absence of large-scale, prospective, observational studies.
Lauris Gastaud - One of the best experts on this subject based on the ideXlab platform.
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Central nervous system involvement by malignant cells is a rare complication of Waldenström Macroglobulinemia, and this clinicopathological entity is referred to as the Bing-Neel syndrome. There is currently no consensus on the diagnostic criteria, t
Haematologica, 2015Co-Authors: Laure Simon, Richard Lemal, Aikaterini Fitsiori, Jehan Dupuis, Benjamin Carpentier, Laurys Boudin, Anne Corby, Lauris Gastaud, Thérèse Aurran-schleinitz, Alexis TalbotAbstract:Central nervous system involvement by malignant cells is a rare complication of Waldenström Macroglobulinemia, and this clinicopathological entity is referred to as the Bing-Neel syndrome. There is currently no consensus on the diagnostic criteria, therapeutic approaches and response evaluation for this syndrome. In this series, we retrospectively analyzed 44 French patients with Bing-Neel syndrome. Bing-Neel syndrome was the first manifestation of Waldenström Macroglobulinemia in 36% of patients. When Waldenström Macroglobulinemia was diagnosed prior to Bing-Neel syndrome, the median time interval between this diagnosis and the onset of Bing-Neel syndrome was 8.9 years. This study highlights the possibility of the occurrence of Bing-Neel syndrome without any other evidence of progression of Waldenström Macroglobulinemia. The clinical presentation was heterogeneous without any specific signs or symptoms. Biologically, the median lymphocyte count in the cerebrospinal fluid was 31/mm(3). Magnetic resonance imaging revealed abnormalities in 78% of the cases. The overall response rate after first-line treatment was 70%, and the overall survival rate after the diagnosis of Bing-Neel syndrome was 71% at 5 years. Altogether, these results suggest that Bing-Neel syndrome should be considered in the context of any unexplained neurological symptoms associated with Waldenström Macroglobulinemia. The diagnostic approach should be based on cerebrospinal fluid analysis and magnetic resonance imaging of the brain and spinal axis. It still remains difficult to establish treatment recommendations or prognostic factors in the absence of large-scale, prospective, observational studies.
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bing neel syndrome a rare complication of waldenstrom Macroglobulinemia analysis of 44 cases and review of the literature a study on behalf of the french innovative leukemia organization filo
Haematologica, 2015Co-Authors: Laurence Simon, Richard Lemal, Aikaterini Fitsiori, Jehan Dupuis, Benjamin Carpentier, Laurys Boudin, Anne Corby, Therese Aurranschleinitz, Lauris Gastaud, Alexis TalbotAbstract:Central nervous system involvement by malignant cells is a rare complication of Waldenstrom Macroglobulinemia, and this clinicopathological entity is referred to as the Bing-Neel syndrome. There is currently no consensus on the diagnostic criteria, therapeutic approaches and response evaluation for this syndrome. In this series, we retrospectively analyzed 44 French patients with Bing-Neel syndrome. Bing-Neel syndrome was the first manifestation of Waldenstrom Macroglobulinemia in 36% of patients. When Waldenstrom Macroglobulinemia was diagnosed prior to Bing-Neel syndrome, the median time interval between this diagnosis and the onset of Bing-Neel syndrome was 8.9 years. This study highlights the possibility of the occurrence of Bing-Neel syndrome without any other evidence of progression of Waldenstrom Macroglobulinemia. The clinical presentation was heterogeneous without any specific signs or symptoms. Biologically, the median lymphocyte count in the cerebrospinal fluid was 31/mm3. Magnetic resonance imaging revealed abnormalities in 78% of the cases. The overall response rate after first-line treatment was 70%, and the overall survival rate after the diagnosis of Bing-Neel syndrome was 71% at 5 years. Altogether, these results suggest that Bing-Neel syndrome should be considered in the context of any unexplained neurological symptoms associated with Waldenstrom Macroglobulinemia. The diagnostic approach should be based on cerebrospinal fluid analysis and magnetic resonance imaging of the brain and spinal axis. It still remains difficult to establish treatment recommendations or prognostic factors in the absence of large-scale, prospective, observational studies.
