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Immunodeficiency cll

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https://www.readbyqxmd.com/read/29644723/a-french-observational-study-describing-the-use-of-human-polyvalent-immunoglobulins-in-hematological-malignancy-associated-secondary-immunodeficiency
#1
Omar Benbrahim, Jean-François Viallard, Sylvain Choquet, Bruno Royer, Frédéric Bauduer, Olivier Decaux, Jean-Charles Crave, Yann Fardini, Pierre Clerson, Vincent Levy
OBJECTIVE: To describe the characteristics of patients suffering from secondary immunodeficiencies (SID) associated with hematological malignancies (HM), who started immunoglobulins replacement therapy (IgRT), physicians' expectations regarding IgRT and IgRT modalities. METHODS: Non-interventional, prospective French cross-sectional study. RESULTS: The analysis included 231 patients (66±12 years old) suffering from multiple myeloma (MM) (N=64), chronic lymphoid leukemia (CLL) (N=84), aggressive non-Hodgkin B-cell lymphoma (aNHL) (N=32), indolent NHL (N=39), acute leukemia (N=6), Hodgkin disease (N=6)...
April 12, 2018: European Journal of Haematology
https://www.readbyqxmd.com/read/29571669/chronic-lymphocytic-leukemia-and-infection-risk-in-the-era-of-targeted-therapies-linking-mechanisms-with-infections
#2
REVIEW
Talal Hilal, Juan C Gea Banacloche, Jose F Leis
Chronic lymphocytic leukemia (CLL) is the most common adult leukemia in the world. Patient with CLL are at particular risk for infections due to inherent disease-related immune dysfunction in addition to the effect of certain systemic therapies on the immune system. The advent of B-cell receptor (BCR) inhibitors such as ibrutinib and idelalisib has led to a practice change that utilizes these targeted agents in the treatment of CLL, either in place of chemoimmunotherapy (CIT) or in later line settings. In this paper, we review the pathophysiology of immune dysfunction in CLL, the spectrum of immunodeficiency with the various therapeutic agents along with prevention strategies with a focus on targeted therapies...
March 16, 2018: Blood Reviews
https://www.readbyqxmd.com/read/29455639/role-of-bruton-s-tyrosine-kinase-in-b-cells-and-malignancies
#3
REVIEW
Simar Pal Singh, Floris Dammeijer, Rudi W Hendriks
Bruton's tyrosine kinase (BTK) is a non-receptor kinase that plays a crucial role in oncogenic signaling that is critical for proliferation and survival of leukemic cells in many B cell malignancies. BTK was initially shown to be defective in the primary immunodeficiency X-linked agammaglobulinemia (XLA) and is essential both for B cell development and function of mature B cells. Shortly after its discovery, BTK was placed in the signal transduction pathway downstream of the B cell antigen receptor (BCR). More recently, small-molecule inhibitors of this kinase have shown excellent anti-tumor activity, first in animal models and subsequently in clinical studies...
February 19, 2018: Molecular Cancer
https://www.readbyqxmd.com/read/29112013/cancer-therapy-associated-lymphoproliferative-disorders-an-under-recognized-type-of-immunodeficiency-associated-lymphoproliferative-disorder
#4
Sergio Pina-Oviedo, Roberto N Miranda, L Jeffrey Medeiros
We describe the clinicopathologic features of 17 patients who had a hematologic malignancy of various types, were treated, and subsequently developed a lymphoproliferative disorder (LPD). There were 10 men and 7 women with a median age of 59 years (range, 36 to 83 y). The primary hematologic neoplasms included: 5 chronic lymphocytic leukemia/small lymphocytic lymphoma, 3 plasma cell myeloma, 2 acute monoblastic leukemia, and 1 case each of mixed-phenotype acute leukemia, chronic myeloid leukemia, splenic marginal zone lymphoma, follicular lymphoma, mantle cell lymphoma, T-cell prolymphocytic leukemia, and peripheral T-cell lymphoma...
January 2018: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28972490/phagocytosis-and-expression-of-fcg-receptors-and-cd180-on-monocytes-in-chronic-lymphocytic-leukemia
#5
T Tsertsvadze, N Mitskevich, A Bilanishvili, D Girdaladze, N Porakishvili
Chronic lymphocytic leukaemia (CLL) is a lymphoproliferative disease characterised by accumulation of monoclonal CD19+CD5+CD23+ lymphocytes in the peripheral blood and bone marrow. CLL is the most common type of the adult leukemia in the Western world. The disease is incurable, albeit there are new molecular and immunotherapy methods currently available in conjunction with chemotherapy, leading to the "precision therapy". The majority of immunotherapeutic approaches are based on the ability of therapeutic antibodies (Rituximab, Alemtuzumab) to mobilize anti-tumour potential of the Natural Killer cells and macrophages/monocytes through their Fcg-receptors (FcγR)...
September 2017: Georgian Medical News
https://www.readbyqxmd.com/read/28623673/a-prominent-lactate-peak-as-a-potential-key-magnetic-resonance-spectroscopy-mrs-feature-of-progressive-multifocal-leukoencephalopathy-pml-spectrum-pattern-observed-in-three-patients
#6
Duško Kozić, Mladen Bjelan, Jasmina Boban, Jelena Ostojić, Vesna Turkulov, Aleksandar Todorović, Slobodanka Lemajić-Komazec, Snežana Brkić
Progressive multifocal leukoencephalopathy (PML) is a rare, often fatal, opportunistic infection, associated with demyelinating process. PML is caused by John Cunningham (JC) polyomavirus, and predominantly affects patients with human immunodeficiency virus (HIV) infection or other immunocompromised patients. The purpose of this study was to determine the role of magnetic resonance spectroscopy (MRS) in establishing the diagnosis of PML. MRS with long and short echo time was performed in two patients with PML associated with HIV infection and in one PML patient associated with chronic lymphocytic leukemia...
November 20, 2017: Bosnian Journal of Basic Medical Sciences
https://www.readbyqxmd.com/read/28385786/host-virus-and-pneumococcus-specific-immune-responses-in-high-count-monoclonal-b-cell-lymphocytosis-and-chronic-lymphocytic-leukemia-implications-for-disease-progression
#7
Ignacio Criado, Santiago Muñoz-Criado, Arancha Rodríguez-Caballero, Wendy G Nieto, Alfonso Romero, Paulino Fernández-Navarro, Miguel Alcoceba, Teresa Contreras, Marcos González, Alberto Orfao, Julia Almeida
Patients diagnosed with chronic lymphocytic leukemia (CLL) display a high incidence of infections due to an associated immunodeficiency that includes hypogammaglobulinemia. A higher risk of infections has also been recently reported for high-count monoclonal B-cell lymphocytosis, while no information is available in low-count monoclonal B-cell lymphocytosis. Here, we evaluated the status of the humoral immune system in patients with chronic lymphocytic leukemia (n=58), as well as in low- (n=71) and high- (n=29) count monoclonal B-cell lymphocytosis versus healthy donors (n=91)...
July 2017: Haematologica
https://www.readbyqxmd.com/read/28202953/fully-human-cd19-specific-chimeric-antigen-receptors-for-t-cell-therapy
#8
COMPARATIVE STUDY
D Sommermeyer, T Hill, S M Shamah, A I Salter, Y Chen, K M Mohler, S R Riddell
Impressive results have been achieved by adoptively transferring T-cells expressing CD19-specific CARs with binding domains from murine mAbs to treat B-cell malignancies. T-cell mediated immune responses specific for peptides from the murine scFv antigen-binding domain of the CAR can develop in patients and result in premature elimination of CAR T-cells increasing the risk of tumor relapse. As fully human scFv might reduce immunogenicity, we generated CD19-specific human scFvs with similar binding characteristics as the murine FMC63-derived scFv using human Ab/DNA libraries...
October 2017: Leukemia: Official Journal of the Leukemia Society of America, Leukemia Research Fund, U.K
https://www.readbyqxmd.com/read/28180067/-hemophagocytic-lymphohistiocytosis-after-ebv-reactivation-and-ibrutinib-treatment-in-relapsed-refractory-chronic-lymphocytic-leukemia
#9
Maurizio Cavallari, Maria Ciccone, Simonetta Falzoni, Francesco Cavazzini, Luca Formigaro, Francesco Di Virgilio, Antonella Rotola, Gian Matteo Rigolin, Antonio Cuneo
Hemophagocytic Lymphohistiocytosis (HLH) is a rare syndrome characterized by ineffective T-cell and NK response. We report the clinical course of a patient with relapsed CLL who developed acute symptoms soon after starting ibrutinib. Hyperpyrexia, splenomegaly, hyperferritinemia, hypertriglyceridemia, cytopenias, and a typical cytokine pattern, i.e. high interleukin (IL)-6, IL10 and IL18, were consistent with a diagnosis of HLH. Coexistent Epstein Barr virus reactivation was documented. Ibrutinib-induced impairment of NK degranulation, associated with EBV reactivation and CLL-related immunodeficiency may have contributed to the development of HLH in our patient...
2017: Leukemia Research Reports
https://www.readbyqxmd.com/read/27737845/cll-an-acquired-immunodeficiency-disease
#10
COMMENT
Clive S Zent
No abstract text is available yet for this article.
October 13, 2016: Blood
https://www.readbyqxmd.com/read/27688087/b-lymphoblastic-leukemia-in-a-patient-with-chronic-lymphocytic-leukemia-sequential-development-of-biclonal-b-cell-neoplasms-over-a-23-year-period-in-a-single-individual
#11
Bin Wu, Kimberly Ingersoll, Catherine Rehder, Endi Wang
Chronic lymphocytic leukemia (CLL) is an indolent mature B-cell neoplasm. During a prolonged disease course, a secondary B-cell neoplasm may arise in some patients, the most common example being the clonal evolution of CLL to diffuse large B-cell lymphoma, which is referred to as Richter transformation. Secondary de novo mature B-cell neoplasms arising in a patient with pre-existing CLL have been described; however, B-lymphoblastic leukemia (B-ALL) developing in untreated CLL is rare, and its clonal relationship to the primary neoplasm has been an interesting issue...
November 2016: Pathology, Research and Practice
https://www.readbyqxmd.com/read/27597852/challenges-in-the-role-of-gammaglobulin-replacement-therapy-and-vaccination-strategies-for-hematological-malignancy
#12
REVIEW
Silvia Sánchez-Ramón, Fatima Dhalla, Helen Chapel
Patients with chronic lymphocytic leukemia (CLL) and multiple myeloma (MM) are prone to present with antibody production deficits associated with recurrent or severe bacterial infections that might benefit from human immunoglobulin (Ig) (IVIg/SCIg) replacement therapy. However, the original IVIg trial data were done before modern therapies were available, and the current indications do not take into account the shift in the immune situation of current treatment combinations and changes in the spectrum of infections...
2016: Frontiers in Immunology
https://www.readbyqxmd.com/read/27450516/uncommon-structural-motifs-dominate-the-antigen-binding-site-in-human-autoantibodies-reactive-with-basement-membrane-collagen
#13
Mary H Foster, Elizabeth S Buckley, Benny J Chen, Kwan-Ki Hwang, Amy G Clark
Autoantibodies mediate organ destruction in multiple autoimmune diseases, yet their origins in patients remain poorly understood. To probe the genetic origins and structure of disease-associated autoantibodies, we engrafted immunodeficient mice with human CD34+ hematopoietic stem cells and immunized with the non-collagenous-1 (NC1) domain of the alpha3 chain of type IV collagen. This antigen is expressed in lungs and kidneys and is targeted by autoantibodies in anti-glomerular basement membrane (GBM) nephritis and Goodpasture syndrome (GPS), prototypic human organ-specific autoimmune diseases...
August 2016: Molecular Immunology
https://www.readbyqxmd.com/read/27402426/secondary-immunodeficiency-in-lymphoproliferative-malignancies
#14
REVIEW
Vanda Friman, Ola Winqvist, Cecilie Blimark, Petra Langerbeins, Helen Chapel, Fatima Dhalla
Secondary immunodeficiencies occur as a consequence of various diseases, including hematological malignancies, and the use of pharmacological therapies, such as immunosuppressive, anti-inflammatory, and biological drugs. Infections are the main cause of morbidity and mortality in multiple myeloma (MM) and chronic lymphocytic leukemia (CLL) patients. Recent advances in treatment have prolonged the duration of remission and the time between relapse phases in MM and CLL patients. However, managing multiple relapses and the use of salvage therapies can lead to cumulative immunosuppression and a higher risk of infections...
September 2016: Hematological Oncology
https://www.readbyqxmd.com/read/27208570/mature-b-cell-neoplasms-retrospective-analysis-of-93-cases-diagnosed-between-2011-and-2014-in-a-university-hospital-in-southern-brazil
#15
Chandra Chiappin Cardoso, Ana Carolina Rabello de Moraes, Joanita Angela Gonzaga Del Moral, Maria Claudia Santos-Silva
BACKGROUND: According to the 2008 World Health Organization classification, mature B-cell neoplasms are a heterogeneous group of diseases that include B-cell lymphomas and plasma cell disorders. These neoplasms can have very different clinical behaviors, from highly aggressive to indolent, and therefore require diverse treatment strategies. OBJECTIVE: The aim of this study was to assess the profile of 93 patients diagnosed with mature B-cell neoplasms monitored between 2011 and 2014...
April 2016: Revista Brasileira de Hematologia e Hemoterapia
https://www.readbyqxmd.com/read/27197296/comprehensive-evaluation-of-medical-conditions-associated-with-risk-of-non-hodgkin-lymphoma-using-medicare-claims-medwas
#16
Eric A Engels, Ruth Parsons, Caroline Besson, Lindsay M Morton, Lindsey Enewold, Winnie Ricker, Elizabeth L Yanik, Hannah Arem, April A Austin, Ruth M Pfeiffer
BACKGROUND: Certain medical conditions affect risk of non-Hodgkin lymphoma (NHL), but the full range of associations is unknown. We implemented a novel method ("medical condition-wide association study," MedWAS) to comprehensively evaluate medical risk factors for NHL documented in administrative health claims. METHODS: Using Surveillance, Epidemiology, and End Results (SEER)-Medicare data, we conducted a case-control study comparing NHL cases [N = 52,691, age 66+ years, with five subtypes: chronic lymphocytic leukemia/small lymphocytic lymphoma, diffuse large B-cell lymphoma (DLBCL), follicular lymphoma, marginal zone lymphoma (MZL), T-cell lymphoma (TCL)] to controls (N = 200,000)...
July 2016: Cancer Epidemiology, Biomarkers & Prevention
https://www.readbyqxmd.com/read/27046033/acute-orbital-syndrome-in-herpes-zoster-ophthalmicus-clinical-features-of-7-cases
#17
Jenny Temnogorod, Renelle Pointdujour-Lim, Ronald Mancini, Shu-Hong Chang, Richard C Allen, Roman Shinder
PURPOSE: To report a series of patients with herpes zoster ophthalmicus and associated acute orbital syndrome with corresponding radiographic findings. METHODS: Medical records of 7 patients with herpes zoster ophthalmicus with acute orbital findings were reviewed. Clinical presentation, radiography, and treatment outcomes were assessed. RESULTS: One man and 6 women with a median age of 70 years (range 47-84) presented with herpes zoster ophthalmicus with acute clinical orbital signs...
May 2017: Ophthalmic Plastic and Reconstructive Surgery
https://www.readbyqxmd.com/read/26951929/agonist-mediated-activation-of-sting-induces-apoptosis-in-malignant-b-cells
#18
Chih-Hang Anthony Tang, Joseph A Zundell, Sujeewa Ranatunga, Cindy Lin, Yulia Nefedova, Juan R Del Valle, Chih-Chi Andrew Hu
Endoplasmic reticulum (ER) stress responses through the IRE-1/XBP-1 pathway are required for the function of STING (TMEM173), an ER-resident transmembrane protein critical for cytoplasmic DNA sensing, IFN production, and cancer control. Here we show that the IRE-1/XBP-1 pathway functions downstream of STING and that STING agonists selectively trigger mitochondria-mediated apoptosis in normal and malignant B cells. Upon stimulation, STING was degraded less efficiently in B cells, implying that prolonged activation of STING can lead to apoptosis...
April 15, 2016: Cancer Research
https://www.readbyqxmd.com/read/26896909/asymptomatic-diffuse-encephalitic-cerebral-toxoplasmosis-in-a-woman-with-systemic-lupus-erythematosus
#19
Diana Murro, Jorge Novo, Leonidas Arvanitis
Classic cerebral toxoplasmosis typically presents with neurologic symptoms such as seizures and mental status changes and histological examination shows focal lesions with necrosis. However, in the diffuse "encephalitic" form, patients are asymptomatic with diffuse, inflammatory, non-necrotic lesions. Asymptomatic diffuse "encephalitic" toxoplasmosis has been reported only in four acquired immunodeficiency syndrome patients and one human immunodeficiency virus (HIV) negative patient with chronic lymphocytic leukemia...
July 2016: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
https://www.readbyqxmd.com/read/26892465/synergism-between-the-mtor-inhibitor-rapamycin-and-fak-down-regulation-in-the-treatment-of-acute-lymphoblastic-leukemia
#20
Pei-Jie Shi, Lu-Hong Xu, Kang-Yu Lin, Wen-jun Weng, Jian-Pei Fang
BACKGROUND: Acute lymphoblastic leukemia (ALL) is an aggressive malignant disorder of lymphoid progenitor cells in both children and adults. Although improvements in contemporary therapy and development of new treatment strategies have led to dramatic increases in the cure rate in children with ALL, the relapse rate remains high and the prognosis of relapsed childhood ALL is poor. Molecularly targeted therapies have emerged as the leading treatments in cancer therapy. Multi-cytotoxic drug regimens have achieved success, yet many studies addressing targeted therapies have focused on only one single agent...
February 18, 2016: Journal of Hematology & Oncology
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