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Immunodeficiency cll

Clive S Zent
No abstract text is available yet for this article.
October 13, 2016: Blood
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...
September 22, 2016: Pathology, Research and Practice
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
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
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
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
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
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...
April 4, 2016: Ophthalmic Plastic and Reconstructive Surgery
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
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
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...
2016: Journal of Hematology & Oncology
Anna Maciejewski-Duval, Floriane Meuris, Alexandre Bignon, Marie-Laure Aknin, Karl Balabanian, Laurence Faivre, Marlène Pasquet, Vincent Barlogis, Claire Fieschi, Christine Bellanné-Chantelot, Jean Donadieu, Géraldine Schlecht-Louf, Viviana Marin-Esteban, Françoise Bachelerie
GATA2 deficiency-formerly described as MonoMAC syndrome; dendritic cells, monocytes, B cells, and natural killer cell deficiency; familial myelodysplastic syndrome/acute myeloid leukemia; or Emberger syndrome-encompasses a range of hematologic and nonhematologic anomalies, mainly characterized by monocytopenia, B lymphopenia, natural killer cell cytopenia, neutropenia, immunodeficiency, and a high risk of developing acute myeloid leukemia. Herein, we present 7 patients with GATA2 deficiency recruited into the French Severe Chronic Neutropenia Registry, which enrolls patients with all kinds of congenital neutropenia...
June 2016: Journal of Leukocyte Biology
N Rockwood, L Cook, H Kagdi, S Basnayake, C R M Bangham, A L Pozniak, G P Taylor
Human immunodeficiency virus type-1 (HIV-1) and human T lymphotropic virus type-1 (HTLV-1) infections have complex effects on adaptive immunity, with specific tropism for, but contrasting effects on, CD4 T lymphocytes: depletion with HIV-1, proliferation with HTLV-1. Impaired T lymphocyte function occurs early in HIV-1 infection but opportunistic infections (OIs) rarely occur in the absence of CD4 lymphopenia. In the unusual case where a HIV-1 infected individual with a high CD4 count presents with recurrent OIs, a clinician is faced with the possibility of a second underlying comorbidity...
December 2015: Medicine (Baltimore)
Nikolaos Kritikos, Dimitrios Priftakis, Stavros Stavrinides, Stefanos Kleanthous, Eleni Sarafianou
Merkel cell carcinoma (MCC) is a rare and aggressive type of neuroendocrine cancer of the skin. It predominantly affects the elderly, with a predilection for the sun-exposed skin of the head and neck. Risk factors include immune-suppressing diseases, such as human immunodeficiency virus (HIV), chronic lymphocytic leukemia and multiple myeloma, organ transplantation, and the presence of the newly-identified Merkel cell polyomavirus (MCPyV). Diagnosis is based on pathological findings, primarily the immunohistochemical determination of cytokeratin 20 positivity...
September 2015: Oncology Letters
Christine Y Louie, Michael A DiMaio, Karen E Matsukuma, Steven E Coutre, Gerald J Berry, Teri A Longacre
Idelalisib is a highly specific small-molecule phosphoinositide-3-kinase δ inhibitor that was recently approved by the Food and Drug Administration for the treatment of chronic lymphocytic leukemia/small lymphocytic lymphoma and follicular lymphoma. The known side effects of idelalisib include severe diarrhea and colitis. Here we report the histologic findings in idelalisib-associated enterocolitis in 11 patients with chronic lymphocytic leukemia or follicular lymphoma receiving idelalisib over a 5-year period (2011 to 2015) at our institution...
December 2015: American Journal of Surgical Pathology
Jerome Paggetti, Franziska Haderk, Martina Seiffert, Bassam Janji, Ute Distler, Wim Ammerlaan, Yeoun Jin Kim, Julien Adam, Peter Lichter, Eric Solary, Guy Berchem, Etienne Moussay
Exosomes derived from solid tumor cells are involved in immune suppression, angiogenesis, and metastasis, but the role of leukemia-derived exosomes has been less investigated. The pathogenesis of chronic lymphocytic leukemia (CLL) is stringently associated with a tumor-supportive microenvironment and a dysfunctional immune system. Here, we explore the role of CLL-derived exosomes in the cellular and molecular mechanisms by which malignant cells create this favorable surrounding. We show that CLL-derived exosomes are actively incorporated by endothelial and mesenchymal stem cells ex vivo and in vivo and that the transfer of exosomal protein and microRNA induces an inflammatory phenotype in the target cells, which resembles the phenotype of cancer-associated fibroblasts (CAFs)...
August 27, 2015: Blood
Mateusz Marek Polaczek, Jacek Zych, Lucyna Opoka, Beata Maksymiuk, Kazimierz Roszkowski-Sliż
Patients with chronic lymphocytic leukemia or non-Hodgkin’s lymphoma are at risk of infectious diseases of respiratory system because of immunodeficiency. Occurrence of organizing pneumonia in leukemic patients is most commonly correlated with bone marrow transplant or treatment with antimitotic agents. There have been only four reported cases of organizing pneumonia related solitarily to leukemia or lymphoma. We present a case of 65-year old gentlemen, diagnosed 8 months earlier with B-cell chronic lymphocytic leukemia with no previous hematologic treatment, who presented symptoms of persistent pneumonia with no significant reaction to antibiotics...
2015: Pneumonologia i Alergologia Polska
Javier Chinen, Luigi D Notarangelo, William T Shearer
Genetic identification of immunodeficiency syndromes has become more efficient with the availability of whole-exome sequencing, expediting the identification of relevant genes and complementing traditional linkage analysis and homozygosity mapping. New genes defects causing immunodeficiency include phophoglucomutase 3 (PGM3), cytidine 5' triphosphate synthase 1 (CTPS1), nuclear factor κB-inducing kinase (NIK), cytotoxic T lymphocyte-associated antigen 4 (CTLA4), B-cell chronic lymphocytic leukemia/lymphoma 10 (BCL10), phosphoinositide-3 kinase regulatory subunit 1 (PIK3R1), IL21, and Jagunal homolog 1 (JAGN1)...
May 2015: Journal of Allergy and Clinical Immunology
Jan Verner, Martin Trbusek, Jana Chovancova, Zuzana Jaskova, Mojmir Moulis, Frantisek Folber, Roman Halouzka, Jiri Mayer, Sarka Pospisilova, Michael Doubek
Xenograft models represent a promising tool to study the pathogenesis of hematological malignancies. To establish a reliable and appropriate in vivo model of aggressive human B-cell leukemia and lymphoma we xenotransplanted four p53-mutated cell lines and one ATM-mutated cell line into immunodeficient NOD/SCID IL2Rγ-null mice. The cell lines MEC-1, SU-DHL-4, JEKO-1, REC-1, and GRANTA-519 were transplanted intraperitoneally or subcutaneously and the engraftment was investigated using immunohistochemistry and flow cytometry...
2015: Leukemia & Lymphoma
Ricardo García-Muñoz, Jesús Feliu, Luis Llorente
The fundamental task of the immune system is to protect the individual from infectious organisms without serious injury to self. The essence of acquired immunity is molecular self/non self discrimination. Chronic lymphocytic leukemia is characterized by a global failure of immune system that begins with the failure of immunological tolerance mechanisms (autoimmunity) and finish with the incapacity to response to non-self antigens (immunodeficiency). Immunological tolerance mechanisms are involved in chronic lymphocytic leukemia (CLL) development...
January 2015: Journal of Autoimmunity
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