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Waldenstrom macroglobulinemia

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https://www.readbyqxmd.com/read/29326801/waldenstrom-s-macroglobulinemia-an-update
#1
REVIEW
Maddalena Mazzucchelli, Anna Maria Frustaci, Marina Deodato, Roberto Cairoli, Alessandra Tedeschi
Waldenstrom Macroglobulinemia is a rare lymphoproliferative disorder with distinctive clinical features. Diagnostic and prognostic characterisation in WM significantly changed with the discovery of two molecular markers: MYD88 and CXCR4. Mutational status of these latter influences both clinical presentation and prognosis and demonstrated therapeutic implications. Treatment choice in Waldenstrom disease is strictly guided by patients age and characteristics, specific goals of therapy, the necessity for rapid disease control, the risk of treatment-related neuropathy, disease features, the risk of immunosuppression or secondary malignancies and potential for future autologous stem cell transplantation...
2018: Mediterranean Journal of Hematology and Infectious Diseases
https://www.readbyqxmd.com/read/29318955/case-report-of-igm-multiple-myeloma-diagnosing-a-rare-hematologic-entity
#2
Fernando Javier Bonilla-Valentín, Javier Cerra, William Cáceres-Perkins, Melissa Alsina
IgM multiple myeloma is an exceedingly rare hematologic entity comprising only less than 0.5% of multiple myeloma cases. Given the rarity of this disorder, it makes it a challenge to differentiate from other more prevalent hematologic disorders like Waldenstrom macroglobulinemia. These 2 diseases have the common finding of an IgM monoclonal gammopathy and distinguishing between these 2 diagnoses is of great importance given that therapy and prognosis differ significantly. This report illustrates the case of a 64-year-old man who presented with IgM lambda monoclonal gammopathy in whom signs, symptoms, laboratories, and imaging were initially thought to be consistent with Waldenstrom macroglobulinemia...
January 2018: Cancer Control: Journal of the Moffitt Cancer Center
https://www.readbyqxmd.com/read/29296899/what-should-be-the-goal-of-therapy-for-waldenstr%C3%A3-m-macroglobulinemia-patients-complete-response-should-be-the-goal-of-therapy
#3
Steven P Treon, Jorge J Castillo
Publisher's Note:This article has a companion.
November 28, 2017: Blood Advances
https://www.readbyqxmd.com/read/29280186/ibrutinib-discontinuation-in-waldenstr%C3%A3-m-macroglobulinemia-etiologies-outcomes-and-igm-rebound
#4
Joshua N Gustine, Kirsten Meid, Toni Dubeau, Patricia Severns, Zachary R Hunter, Yang Guang, Lian Xu, Steven P Treon, Jorge J Castillo
Ibrutinib is the first approved therapy for symptomatic patients with Waldenström macroglobulinemia (WM). The reasons for discontinuing ibrutinib and subsequent outcomes have not been previously evaluated in WM patients. We therefore conducted a retrospective review of 189 WM patients seen at our institution who received treatment with ibrutinib, of whom 51 (27%) have discontinued therapy. Reasons for discontinuation include: disease progression (n=27; 14%), toxicity (n=15; 8%), non-response (n=5; 3%), and other unrelated reasons (n=4; 2%)...
December 27, 2017: American Journal of Hematology
https://www.readbyqxmd.com/read/29225979/waldenstrom-s-macroglobulinemia-a-report-of-two-cases-one-with-severe-retinopathy-and-one-with-renal-failure
#5
Naoko Kudo, Masakatsu Usui, Yukiharu Nakabo, Ken-Ichi Yoshida, Kenji Miki, Takashi Osafune, Keisuke Nishimura, Shinsaku Imashuku
We report here two cases of Waldenstrom's macroglobulinemia (WM), one with central nervous system (CNS) symptoms and severe retinopathy and one with renal failure. In both cases, the serum IgM levels exceeded 3,000 mg/dL and monoclonal IgM-kappa was observed in the blood. At onset, Case 1, a 63-year-old female, developed CNS symptoms-namely, drowsiness and syncope. Case 2, a 58-year-old male, had nausea and dysgeusia on admission associated with renal failure, which is quite rare in patients with WM. Both patients exhibited hyperviscosity-related retinopathy, but it was particularly severe in Case 1: she suddenly lost her vision after admission...
2017: Case Reports in Hematology
https://www.readbyqxmd.com/read/29222280/toward-personalized-treatment-in-waldenstr%C3%A3-m-macroglobulinemia
#6
REVIEW
Jorge J Castillo, Steven P Treon
Waldenström macroglobulinemia (WM) is a rare lymphoma with 1000 to 1500 new patients diagnosed per year in the United States. Patients with WM can experience prolonged survival times, which seem to have increased in the last decade, but relapse is inevitable. The identification of recurrent mutations in the MYD88 and CXCR4 genes has opened avenues of research to better understand and treat patients with WM. These developments are giving way to personalized treatment approaches for these patients, focusing on increasing depth and duration of response alongside lower toxicity rates...
December 8, 2017: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/29212343/nonbacterial-thrombotic-endocarditis-involving-all-four-cardiac-valves
#7
Sachiko Kawada, Mitsuhito Kuriyama, Yasuhiro Kotani, Shou Tsushima, Atsushi Tanabe, Yukio Kioka
This report describes a case of nonbacterial thrombotic endocarditis caused by Waldenström macroglobulinemia, with diffuse endocardial lesions and involvement of all 4 cardiac valves. A 77-year-old man presented with heart failure due to severe regurgitation of all 4 cardiac valves; surgical repair using bioprosthetic valves was indicated. A pathological study revealed fibrin-triggered thrombus formation that confirmed the diagnosis of nonbacterial thrombotic endocarditis. In cases of nonbacterial thrombotic endocarditis, the underlying cause should be investigated...
January 1, 2017: Asian Cardiovascular & Thoracic Annals
https://www.readbyqxmd.com/read/29203585/safety-and-efficacy-of-selinexor-in-relapsed-or-refractory-multiple-myeloma-and-waldenstrom-s-macroglobulinemia
#8
Christine Chen, David Siegel, Martin Gutierrez, Meagan Jacoby, Craig C Hofmeister, Nashat Gabrail, Rachid Baz, Morten Mau-Sorensen, Jesus G Berdeja, Michael Savona, Lynn Savoie, Suzanne Trudel, Nuchanan Areethamsirikul, T J Unger, Tami Rashal, Tim Hanke, Michael Kauffman, Sharon Shacham, Donna Reece
Novel therapies are needed for patients with relapsed or refractory multiple myeloma (MM). We conducted a multicenter, phase 1 study in advanced hematological malignancies to assess the safety, efficacy and recommended phase 2 dose (RP2D) of oral selinexor, a selective inhibitor of the nuclear export protein XPO1. In the dose-escalation phase, 25 patients with heavily pretreated MM (22) or Waldenstrom's macroglobulinemia (3) were administered selinexor (3-60 mg/m2) in 8 or 10 doses per 28-day cycle. In the dose-expansion phase, 59 patients with MM received selinexor at 45 or 60 mg/m2 with 20 mg dexamethasone, twice-weekly in 28-day cycles, or selinexor (40 or 60mg flat dose) without corticosteroids in 21 day cycles...
December 4, 2017: Blood
https://www.readbyqxmd.com/read/29201288/cost-effectiveness-analysis-of-ibrutinib-in-patients-with-waldenstr%C3%A3-m-macroglobulinemia-in-italy
#9
Andrea Aiello, Anna D'Ausilio, Roberta Lo Muto, Francesca Randon, Luca Laurenti
Background and Objective: Ibrutinib has recently been approved in Europe for Waldenström Macroglobulinemia (WM) in symptomatic patients who have received at least one prior therapy, or in first-line treatment for patients unsuitable for chemo-immunotherapy. The aim of the study is to estimate the incremental cost-effectiveness ratio (ICER) of ibrutinib in relapse/refractory WM, compared with the Italian current therapeutic pathways (CTP). Methods: A Markov model was adapted for Italy considering the National Health System perspective...
2017: Journal of Market Access & Health Policy
https://www.readbyqxmd.com/read/29183887/how-i-manage-monoclonal-gammopathy-of-undetermined-significance
#10
Ronald S Go, S Vincent Rajkumar
Monoclonal gammopathy of undetermined significance (MGUS) is, in many ways, a unique hematologic entity. Unlike most hematologic conditions in which the diagnosis is intentional and credited to hematologists, the discovery of MGUS is most often incidental and made by non-hematologists. MGUS is considered an obligate precursor to several lymphoplasmacytic malignancies including immunoglobulin light chain amyloidosis, multiple myeloma, and Waldenström macroglobulinemia. Therefore, long-term follow-up is generally recommended...
November 28, 2017: Blood
https://www.readbyqxmd.com/read/29181138/bing-neel-syndrome-illustrative-cases-and-comprehensive-review-of-the-literature
#11
REVIEW
Marzia Varettoni, Irene Defrancesco, Luca Diamanti, Enrico Marchioni, Lisa Maria Farina, Anna Pichiecchio
The Bing-Neel syndrome is a rare neurological complication of Waldenström's Macroglobulinemia which results from a direct involvement of central nervous system by malignant lymphoplasmacytic cells. The clinical suspicion of Bing-Neel syndrome may be overlooked because neurologic symptoms are heterogeneous, nonspecific and sometimes underhand. A definitive diagnosis of Bing-Neel syndrome can be confidently made using brain and spinal cord magnetic resonance imaging as well as histopathology and/or cerebrospinal fluid analysis to confirm the neoplastic infiltration of central nervous system...
2017: Mediterranean Journal of Hematology and Infectious Diseases
https://www.readbyqxmd.com/read/29169431/monoclonal-igm-gammopathy-and-waldenstr%C3%A3-m-s-macroglobulinemia
#12
Alexander Grunenberg, Christian Buske
BACKGROUND: 3.2-3.5% of persons over age 50 have a monoclonal gammopathy. Monoclonal gammopathies have many causes, including cancer. 10-20% of monoclonal gammopathies are of isotype IgM. A systematic approach to the differential diagnosis of IgM gammopathies is essential because of the different therapeutic implications of the various underlying conditions. METHODS: This review is based on pertinent publications retrieved by a selective search in PubMed and current guidelines from Germany and abroad...
November 3, 2017: Deutsches Ärzteblatt International
https://www.readbyqxmd.com/read/29118506/localized-retinal-degeneration-secondary-to-waldenstr%C3%A3-m-s-macroglobulinemia
#13
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
#14
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
#15
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/myd88-mutation-status-does-not-impact-overall-survival-in-waldenstr%C3%A3-m-macroglobulinemia
#16
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 D McPhail, David S Viswanatha, Thomas E Witzig, Ronald S Go, Thomas M Habermann, Francis K Buadi, Angela Dispenzieri, Nelson Leung, Yi Lin, Carrie A Thompson, Suzanne R Hayman, Robert A Kyle, Shaji K Kumar, Prashant Kapoor
Waldenström macroglobulinemia (WM) is an immunoglobulin M-associated lymphoma, with majority of cases demonstrating MYD88 locus alteration, most commonly, MYD88L265P . Owing 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 MYD88L265P mutation. We studied a large cohort of patients with MYD88L265P and MYD88WT 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
#17
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
#18
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
#19
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
#20
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
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