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Lymphoproliferative

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https://www.readbyqxmd.com/read/28231257/targeted-sequencing-identifies-a-novel-sh2d1a-pathogenic-variant-in-a-chinese-family-carrier-screening-and-prenatal-genetic-testing
#1
Jun-Yu Zhang, Song-Chang Chen, Yi-Yao Chen, Shu-Yuan Li, Lan-Lan Zhang, Ying-Hua Shen, Chun-Xin Chang, Yu-Qian Xiang, He-Feng Huang, Chen-Ming Xu
X-linked lymphoproliferative disease type 1 (XLP1) is a rare primary immunodeficiency characterized by a clinical triad consisting of severe EBV-induced hemophagocytic lymphohistiocytosis, B-cell lymphoma, and dysgammaglobulinemia. Mutations in SH2D1A gene have been revealed as the cause of XLP1. In this study, a pregnant woman with recurrence history of birthing immunodeficiency was screened for pathogenic variant because the proband sample was unavailable. We aimed to clarify the genetic diagnosis and provide prenatal testing for the family...
2017: PloS One
https://www.readbyqxmd.com/read/28225293/rituximab-associated-progressive-multifocal-leukoencephalopathy
#2
Juraj Sokol, Lenka Lisá, Jana Zeleňáková, Tomáš Balhárek, Ivana Plameňová, Ján Staško, Peter Kubisz
The definition "Progressive Multifocal Leukoencephalopathy" (PML) was first used in 1958 to describe a fatal demyelinating central nervous system (CNS) disease in patients with lymphoproliferative disorders. In 1971, the virus responsible for the disease was isolated and named John Cunningham virus (JCV). We present a rare case of a 62-year-old male with chronic lymphocytic leukemia and PML. In our work, we discuss the diagnostic and therapeutic challenges and offer suggestions for preventing PML development...
2017: Vnitr̆ní Lékar̆ství
https://www.readbyqxmd.com/read/28219580/use-of-anti-thymocyte-globulin-for-induction-therapy-in-cardiac-transplantation-a-review
#3
V Ruan, L S C Czer, M Awad, M Kittleson, J Patel, F Arabia, F Esmailian, D Ramzy, J Chung, M De Robertis, A Trento, J A Kobashigawa
The most common causes of death after heart transplantation (HTx) include acute rejection and multi-organ failure in the early period and malignancy and cardiac allograft vasculopathy (CAV) in the late period. Polyclonal antibody preparations such as rabbit anti-thymocyte globulin (ATG) may reduce early acute rejection and the later occurrence of CAV after HTx. ATG therapy depletes T cells, modulates adhesion and cell-signaling molecules, interferes with dendritic cell function, and induces B-cell apoptosis and regulatory and natural killer T-cell expansion...
March 2017: Transplantation Proceedings
https://www.readbyqxmd.com/read/28218349/mri-and-18f-fdg-pet-ct-in-a-rare-case-of-early-precursor-b-lymphoblastic-leukaemia-with-bone-involvement-as-initial-manifestation
#4
Angelina Cistaro, Vincenzo La Delfa, Gianpaolo Di Rosa, Maurizio Cogoni, Natale Quartuccio
A 4-year old girl presenting gait difficulty was referred for spine X-ray and Magnetic Resonance Imaging (MRI). MRI showed several diffuse hypointense signals in sacral and lumbar vertebrae. In order to exclude a possible lymphoproliferative disease a 18-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG-PET/CT) was requested. The PET/CT scan confirmed the MRI findings and demonstrated additional findings in the sternum. Therefore, a bone marrow biopsy was performed and a diagnosis of acute lymphoblastic leukaemia - early B type was made...
2017: Nuclear Medicine Review. Central & Eastern Europe
https://www.readbyqxmd.com/read/28216262/efis-lecture-understanding-the-ctla-4-checkpoint-in-the-maintenance-of-immune-homeostasis
#5
REVIEW
Lucy S K Walker
The past 20 years have heralded fascinating developments in the field of CTLA-4 biology. The CTLA-4 protein is a critical negative regulator of T cell immunity and its absence provokes severe lymphoproliferative disease. In a surprising twist, the generation of mixed bone marrow chimeric mice revealed that CTLA-4 predominantly functions in a cell-extrinsic manner, suggesting that CTLA-4 expressed on one cell can modify the behaviour of another cell. This was followed by the demonstration that CTLA-4 is highly expressed in regulatory T cells and can contribute to their suppressive activity...
February 16, 2017: Immunology Letters
https://www.readbyqxmd.com/read/28215641/coexistence-of-age-related-ebv-associated-follicular-hyperplasia-and-large-b-cell-ebv-lymphoma-of-the-elderly-two-distinct-features-of-the-same-t-cell-dysfunction-related-to-senescence
#6
Jean-Baptiste Gibier, Brigitte Bouchindhomme, Romain Dubois, Benedicte Hivert, Nathalie Grardel, Marie-Christine Copin
Age-related EBV-associated lymphoproliferative disorders form a new clinicopathological group. Until recently, this group was associated with diffuse large B-cell lymphoma (DLBCL), characterised by an aggressive clinical presentation and a poor prognosis. Recent findings in Western Caucasian patients, however, suggest that this entity covers a wide spectrum of diseases, ranging from reactive follicular hyperplasia (HR) to DLBCL. We report the case of an 85-year-old Caucasian man showing lymphadenopathy and numerous hypodense lesions of the liver...
December 23, 2016: Pathology, Research and Practice
https://www.readbyqxmd.com/read/28215425/outcomes-of-lung-transplantation-for-infants-and-children-with-genetic-disorders-of-surfactant-metabolism
#7
Whitney B Eldridge, Qunyuan Zhang, Albert Faro, Stuart C Sweet, Pirooz Eghtesady, Aaron Hamvas, F Sessions Cole, Jennifer A Wambach
OBJECTIVE: To compare outcomes of infants and children who underwent lung transplantation for genetic disorders of surfactant metabolism (SFTPB, SFTPC, ABCA3, and NKX2-1) over 2 epochs (1993-2003 and 2004-2015) at St Louis Children's Hospital. STUDY DESIGN: We retrospectively reviewed clinical characteristics, mortality, and short- and long-term morbidities of infants (transplanted at <1 year; n = 28) and children (transplanted >1 year; n = 16) and compared outcomes by age at transplantation (infants vs children) and by epoch of transplantation...
February 16, 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/28210942/how-we-treat-chronic-active-epstein-barr-virus-infection
#8
REVIEW
Akihisa Sawada, Masami Inoue, Keisei Kawa
Chronic active Epstein-Barr virus infection (CAEBV) is a prototype of the EBV-associated T- or NK-cell lymphoproliferative diseases, which also include hypersensitivity to mosquito bites and severe-type hydroavacciniforme. The manifestations of CAEBV are often self-limiting with minimum supportive care or only prednisolone and cyclosporine A with or without etoposide. However, allogeneic hematopoietic stem cell transplantation (HSCT) is the only cure, without which patients with CAEBV die within several years...
February 16, 2017: International Journal of Hematology
https://www.readbyqxmd.com/read/28205564/elevated-serum-interferon-%C3%AE-induced-protein-10-kda-is-associated-with-tafro-syndrome
#9
Noriko Iwaki, Yuka Gion, Eisei Kondo, Mitsuhiro Kawano, Taro Masunari, Hiroshi Moro, Koji Nikkuni, Kazue Takai, Masao Hagihara, Yuko Hashimoto, Kenji Yokota, Masataka Okamoto, Shinji Nakao, Tadashi Yoshino, Yasuharu Sato
Multicentric Castleman disease (MCD) is a heterogeneous lymphoproliferative disorder. It is characterized by inflammatory symptoms, and interleukin (IL)-6 contributes to the disease pathogenesis. Human herpesvirus 8 (HHV-8) often drives hypercytokinemia in MCD, although the etiology of HHV-8-negative MCD is idiopathic (iMCD). A distinct subtype of iMCD that shares a constellation of clinical features including thrombocytopenia (T), anasarca (A), fever (F), reticulin fibrosis (R), and organomegaly (O) has been reported as TAFRO-iMCD, however the differences in cytokine profiles between TAFRO-iMCD and iMCD have not been established...
February 13, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28199343/long-term-treatment-with-abatacept-or-tocilizumab-does-not-increase-epstein-barr-virus-load-in-patients-with-rheumatoid-arthritis-a-three-years-retrospective-study
#10
Nathalie Balandraud, Gaetan Texier, Emmanuel Massy, Olivier Muis-Pistor, Marielle Martin, Isabelle Auger, Marie-Caroline Guzian, Sandrine Guis, Thao Pham, Jean Roudier
BACKGROUND: Epstein-Barr Virus (EBV) is a widely disseminated lymphotropic herpes virus implicated in benign and malignant disorders. In transplant patients, immunosuppressive drugs (cyclosporine) diminish control of EBV replication, potentially leading to lymphoproliferative disorders (LPD). Rheumatoid arthritis (RA) patients have impaired control of EBV infection and have EBV load ten times higher than controls. As post transplant patients, patients with RA have increased risk of developing lymphomas...
2017: PloS One
https://www.readbyqxmd.com/read/28196776/ebv-induced-lymphoproliferative-disorders-in-rheumatic-patients-a-systematic-review-of-the-literature
#11
Alvise Berti, Mara Felicetti, Susanna Peccatori, Roberto Bortolotti, Anna Guella, Paolo Vivaldi, Luca Morelli, Mattia Barabareschi, Giuseppe Paolazzi
OBJECTIVES: Epstein-Barr virus (EBV) is involved in the pathogenesis of approximately 40% of lymphoproliferative disorders (LPDs) arising in patients receiving immunosuppressive treatment (IST) for rheumatic diseases, but data from large cohorts are still not available. We aimed to identify clinicopathological features, management and outcome of this condition. METHODS: We reviewed all published cases of EBV-encoded RNA (EBER)-positive LPDs and included in our analysis one unpublished patient diagnosed in our Hospital...
February 11, 2017: Joint, Bone, Spine: Revue du Rhumatisme
https://www.readbyqxmd.com/read/28195879/fibrin-associated-ebv-positive-large-b-cell-lymphoma-an-indolent-neoplasm-with-features-distinct-from-diffuse-large-b-cell-lymphoma-associated-with-chronic-inflammation
#12
Daniel F Boyer, Penelope A McKelvie, Laurence de Leval, Kerstin L Edlefsen, Young-Hyeh Ko, Zachary A Aberman, Alexandra E Kovach, Aneal Masih, Ha T Nishino, Lawrence M Weiss, Alan K Meeker, Valentina Nardi, Maryknoll Palisoc, Lina Shao, Stefania Pittaluga, Judith A Ferry, Nancy Lee Harris, Aliyah R Sohani
Incidental cases of localized fibrin-associated Epstein-Barr virus (EBV)+ large B-cell proliferations have been described at unusual anatomic sites and have been included in the category of diffuse large B-cell lymphoma associated with chronic inflammation (DLBCL-CI) in the WHO Classification. We describe 12 cases and review the literature to define their clinicopathologic spectrum and compare features with typical cases of DLBCL-CI. Median age was 55.5 years with a M:F ratio of 3. In all 12 cases, the lymphoma was an incidental microscopic finding involving atrial myxomas (n=3), thrombi associated with endovascular grafts (n=3), chronic hematomas (n=2), and pseudocysts (n=4)...
March 2017: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28193515/infliximab-not-associated-with-increased-risk-of-malignancy-or-hemophagocytic-lymphohistiocytosis-in-pediatric-patients-with-inflammatory-bowel-disease
#13
Jeffrey S Hyams, Marla C Dubinsky, Robert N Baldassano, Richard B Colletti, Salvatore Cucchiara, Johanna Escher, William Faubion, John Fell, Benjamin D Gold, Anne Griffiths, Sibylle Koletzko, Subra Kugathasan, James Markowitz, Frank M Ruemmele, Gigi Veereman, Harland Winter, Nicholas Masel, Chu Ri Shin, Kezhen L Tang, Meena Thayu
BACKGROUND AND AIMS: Immunosuppressive therapy for inflammatory bowel disease (IBD) in pediatric patients is thought to increase risk of malignancy and lymphoproliferative disorders, including hemophagocytic lymphohistiocytosis (HLH). We compared unadjusted incidence rates and of malignancy and HLH in pediatric patients with IBD exposed to infliximab compared with patients not exposed to biologics and calculated standardized incidence ratios (SIRs). METHODS: We collected and analyzed data from 5766 participants in a prospective study of long-term outcomes of pediatric patients with IBD (NCT00606346), from 2007 through 30 June 2016...
February 10, 2017: Gastroenterology
https://www.readbyqxmd.com/read/28191298/asymptomatic-epstein-barr-virus-shedding-in-the-urine-of-kidney-transplant-recipients-case-reports-and-review-of-the-literature
#14
Maryam Rahbar, Gholamreza Poormand, Masoud Karkhaneh Mahmoodi, Aboozar Jazayeri, Seyed Mohammad Jazayeri
Epstein-Barr virus (EBV) is associated with a wide range of malignancies and complications like post-transplant lymphoproliferative disorder (PTLD). To suppress active EBV infection in transplant recipients, who are at a heightened risk of developing PTLD, EBV DNAemia monitoring has been recommended. Quantitative multiplex real time polymerase chain reaction (QMRTPCR) offered the advantage of detection of more than one target in the same sample. We present four cases of kidney transplant recipient who were admitted for rising serum creatinine between 9 and 20 months post-transplant with a suspicion of BKV-associated nephropathy...
December 31, 2016: Infectious Disease Reports
https://www.readbyqxmd.com/read/28190860/activated-phosphoinositide-3-kinase-%C3%AE-syndrome-presenting-with-gut-associated-t-cell-lymphoproliferative-disease
#15
Hideto Teranishi, Masataka Ishimura, Yuuki Koga, Katsuhide Eguchi, Motoshi Sonoda, Tetsuko Kobayashi, Satoru Shiraishi, Kentaro Nakashima, Kouji Ikegami, Murasaki Aman, Hidetaka Yamamoto, Hidetoshi Takada, Shouichi Ohga
A 13-year-old boy was admitted to our hospital because of persistent diarrhea, abdominal pain, and bloody stools. The patient had experienced repeated hospitalizations for the treatment of respiratory infections since early childhood. Colonoscopic and pathological studies led to a diagnosis of gut-associated T-cell lymphoproliferative disease (T-cell LPD). Laboratory data showed T-lymphocytopenia (492/µl), increased serum IgG levels (1,984 mg/dl), and low serum antibody titers for specific pathogens. Combined immunodeficiency accompanied by T-LPD suggested the diagnosis of activated PI3Kδ syndrome (APDS)...
2017: [Rinshō Ketsueki] the Japanese Journal of Clinical Hematology
https://www.readbyqxmd.com/read/28188718/-hematopoietic-stem-cells-transplant-in-patients-with-common-variable-immunodeficiency-is-a-therapeutic-option
#16
Julio César Cambray-Gutiérrez, Diana Andrea Herrera-Sánchez, Patricia López-Pérez, Aurora Chávez-García, Marco Antonio Yamazaki-Nakashimada
BACKGROUND: Patients with common variable immunodeficiency show higher incidence of sinopulmonary and gastrointestinal infections, as well as lymphoproliferative and autoimmune diseases. The treatment of choice is replacement therapy with human gamma-globulin. Hematopoietic stem cell transplantation is a non-conventional therapeutic modality. CASE REPORT: Twenty-six-year old woman with no family or hereditary history of primary immune deficiencies or consanguinity, with repeated episodes of otitis, sinusitis, gastroenteritis and bronchitis since childhood...
January 2017: Revista Alergia Mexico: Organo Oficial de la Sociedad Mexicana de Alergia e Inmunología, A.C
https://www.readbyqxmd.com/read/28187925/aggressive-cutaneous-t-cell-lymphomas
#17
Jacqueline M Junkins-Hopkins Md
Cutaneous T cell lymphomas (CTCLs) are heterogeneous, with a prognosis determined in large part by combined clinical, histopathologic, and immunophenotypic features. They are classified under the WHO-EORTC classification of primary cutaneous lymphoma. Whether or not a patient diagnosed with CTCL will experience an aggressive course may not be completely predictable; however, certain subtypes have been proven to be associated with a poor response to therapy and/or short survival. These aggressive subtypes may be diagnosed by certain histologic, immunophenotypic, and clinical features; however, there are benign lymphoproliferative disorders (LPD), such as lymphomatoid papulosis (LyP) that present with biopsy findings that simulate an aggressive lymphoma...
December 24, 2016: Seminars in Diagnostic Pathology
https://www.readbyqxmd.com/read/28185466/-analytical-interference-leading-to-a-diagnosis-of-lymphoproliferative-disorder
#18
I Kurfürstová, T Šálek, E Kadlčková, M Pšenčík
BACKGROUND: Monoclonal immunoglobulins (paraproteins) are produced by B lymphocytes in lymphoproliferative disorders. A single monoclonal immunoglobulin is homogeneous in terms of its structure, and it can occur in human serum at high concentration and cause significant interference in laboratory assays. CASE: We present a case of an 84-year-old man who was admitted to the hospital for progression of dyspnea. Basic laboratory tests showed a serum concentration of conjugated bilirubin, measured using the diazo spectrophotometric method, which was much higher than that of total bilirubin...
2017: Klinická Onkologie: Casopis Ceské a Slovenské Onkologické Spolecnosti
https://www.readbyqxmd.com/read/28182323/ibrutinib-associated-bleeding-pathogenesis-management-and-risk-reduction-strategies
#19
REVIEW
Joseph J Shatzel, Sven R Olson, Derrick L Tao, Owen J T McCarty, Alexey V Danilov, Thomas G DeLoughery
Ibrutinib is an irreversible inhibitor of Bruton's tyrosine kinase (Btk) that has proven to be an effective therapeutic agent for multiple B-cell mediated lymphoproliferative disorders. Ibrutinib, however, carries an increased bleeding risk compared to standard chemotherapy. Bleeding events range from minor mucocutaneous bleeding to life-threatening hemorrhage, due in large part to the effects of ibrutinib on several distinct platelet signaling pathways. There is currently minimal data to guide clinicians regarding the use of ibrutinib in patients at high risk for bleeding or on anticoagulant or antiplatelet therapy...
February 9, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/28181423/next-generation-sequencing-of-mirnas-in-clinical-samples-of-epstein-barr-virus-associated-b-cell-lymphomas
#20
Kouta Sakamoto, Tsuyoshi Sekizuka, Taeko Uehara, Tsunekazu Hishima, Sohtaro Mine, Hitomi Fukumoto, Yuko Sato, Hideki Hasegawa, Makoto Kuroda, Harutaka Katano
Epstein-Barr virus (EBV) encodes 49 microRNAs (miRNAs) in the BART and BHRF1 regions of its genome. Although expression profiles of EBV-encoded miRNAs have been reported for EBV-positive cell lines and nasopharyngeal carcinoma, to date there is little information about total miRNA expression, including cellular and viral miRNAs, in the primary tumors of EBV-associated B-lymphoproliferative disorders. In this study, next-generation sequencing and quantitative real-time reverse transcription-PCR were used to determine the expression profiles of miRNAs in EBV-infected cell lines and EBV-associated B-cell lymphomas, including AIDS-related diffuse large B-cell lymphoma (DLBCL), pyothorax-associated lymphoma, methotrexate-associated lymphoproliferative disorder, EBV-positive DLBCL of the elderly, and Hodgkin lymphoma...
February 9, 2017: Cancer Medicine
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