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https://www.readbyqxmd.com/read/28646630/characteristics-of-systemic-inflammation-in-hepatitis-b-precipitated-aclf-differentiate-it-from-no-aclf
#1
Wei Wu, Huadong Yan, Hong Zhao, Wenjie Sun, Qiao Yang, Jifang Sheng, Yu Shi
BACKGROUND AND AIMS: Patients with severe exacerbation of chronic hepatitis B (SE-CHB) are at risk of developing acute-on-chronic liver failure (ACLF). Systemic inflammation (SI) is a major driver of ACLF. The aim of this study was to identify characteristics of SI in hepatitis B-precipitated-ACLF (HB-ACLF), which may be distinct from No-ACLF patients with SE-CHB. METHODS: Two cohorts of patients with SE-CHB were enrolled in two tertiary hospitals. The associations between circulating leukocyte counts/subsets and ACLF progression and prognoses were analysed in Cohort A...
June 24, 2017: Liver International: Official Journal of the International Association for the Study of the Liver
https://www.readbyqxmd.com/read/28639416/persistently-low-lymphocyte-counts-after-fcr-therapy-for-chronic-lymphocytic-leukemia-are-associated-with-longer-overall-survival
#2
Erel Joffe, N Ariela Arad, Osnat Bairey, Riva Fineman, Rosa Ruchlemer, Naomi Rahimi-Levene, Lev Shvidel, Uri Greenbaum, Ariel Aviv, Tamar Tadmor, Andrei Braester, Neta Goldschmidt, Aaron Polliack, Yair Herishanu
Decreased absolute lymphocyte counts (ALCs) following frontline therapy for chronic lymphocytic leukemia may be associated with disease control, even in patients without evidence of minimal residual disease. We studied the prognostic significance of ALCs during the first year following treatment with fludarabine, cyclophosphamide, and rituximab (FCR). We evaluated 99 patients who achieved a partial response without lymphocytosis (<4.0 × 10(3) cells/μL) or better after FCR. Absolute lymphocyte counts were recorded at 3-, 6-, 9-, and 12-month posttreatment and correlated with overall survival (OS) and event-free survival (EFS)...
June 22, 2017: Hematological Oncology
https://www.readbyqxmd.com/read/28638843/susceptibility-to-cryptococcal-meningoencephalitis-associated-with-idiopathic-cd4-lymphopenia-and-secondary-germline-or-acquired-defects
#3
Anil A Panackal, Lindsey B Rosen, Gulbu Uzel, Michael J Davis, Guowu Hu, Adebowale Adeyemo, Fasil Tekola-Ayele, Andrea Lisco, Christopher Diachok, Jonathan D Kim, Dawn Shaw, Irini Sereti, Jennifer Stoddard, Julie Niemela, Sergio D Rosenzweig, John E Bennett, Peter R Williamson
Idiopathic CD4(+) lymphopenia (ICL) predisposes to opportunistic infections (OIs) but can often remain asymptomatic and does not have a strong association with monogenic mutations. Likewise, cryptococcal meningoencephalitis, the most common OI in ICL, is not strongly associated with monogenic mutations. In this study, we describe 2 patients with ICL plus an additional immune defect: one from an E57K genetic mutation in the nuclear factor-κβ essential modulator, and the other with acquired autoantibodies to granulocyte-macrophage colony-stimulating factor...
2017: Open Forum Infectious Diseases
https://www.readbyqxmd.com/read/28638678/detailed-features-of-hematological-involvement-and-medication-induced-cytopenia-in-systemic-lupus-erythematosus-patients-single-center-results-of-221-patients
#4
Hava Üsküdar Teke, Döndü Üsküdar Cansu, Cengiz Korkmaz
OBJECTIVE: Systemic lupus erythematosus (SLE) may affect a number of systems, with the hematological system being one of the most common. Our aim is to determine the existence of cytopenia at diagnosis or during follow-up of our SLE patients as well as the associated factors. MATERIAL AND METHODS: A cohort of SLE patients that had been followed-up in the Department of Rheumotology from 1998 to 2015 was retrospectively assessed. Clinical and laboratory findings about the patients were recorded...
June 2017: European Journal of Rheumatology
https://www.readbyqxmd.com/read/28633762/usefulness-of-mean-platelet-volume-to-lymphocyte-ratio-for-predicting-angiographic-no-reflow-and-short-term-prognosis-after-primary-percutaneous-coronary-intervention-in-patients-with-st-segment-elevation-myocardial-infarction
#5
Alparslan Kurtul, Sadik Kadri Acikgoz
Primary percutaneous coronary intervention (pPCI) is associated with improved prognosis in patients with ST-segment elevation myocardial infarction (STEMI). However, no-reflow phenomenon limits the benefit of revascularization and predicts adverse outcomes. The specific mechanism for its occurrence is still not entirely clear, and it is believed at present that platelet activation and inflammation play a pivotal role in developing no-reflow. Both increased mean platelet volume (MPV), which is a platelet activation marker, and lymphopenia, which is an inflammation marker, have been linked to adverse events and poor prognosis after STEMI...
May 30, 2017: American Journal of Cardiology
https://www.readbyqxmd.com/read/28622959/a-phase-i-ii-trial-of-panobinostat-in-combination-with-lenalidomide-in-patients-with-relapsed-or-refractory-hodgkin-lymphoma
#6
Joseph J Maly, Beth A Christian, Xiaohua Zhu, Lai Wei, Jennifer L Sexton, Samantha M Jaglowski, Steven M Devine, Todd A Fehniger, Nina D Wagner-Johnston, Mitch A Phelps, Nancy L Bartlett, Kristie A Blum
BACKGROUND: Lenalidomide and panobinostat have shown single-agent efficacy of 14% to 50% and 27% to 58%, respectively, in Hodgkin lymphoma (HL). This phase I/II study was conducted to determine the maximum tolerated dose (MTD), safety, and efficacy of lenalidomide combined with panobinostat in relapsed/refractory HL. PATIENTS AND METHODS: In the phase I trial, previously treated patients with classical or lymphocyte-predominant HL received escalating doses of lenalidomide on days 1 to 21 and panobinostat 3 times a week (TIW) every 28 days...
June 2017: Clinical Lymphoma, Myeloma & Leukemia
https://www.readbyqxmd.com/read/28619037/ifn-%C3%AE-1-with-th17-axis-cytokines-and-ifn-%C3%AE-define-different-subsets-in-systemic-lupus-erythematosus-sle
#7
Vilija Oke, Susanna Brauner, Anders Larsson, Johanna Gustafsson, Agneta Zickert, Iva Gunnarsson, Elisabet Svenungsson
BACKGROUND: Interferon (IFN)-α is thought to have a pivotal role in systemic lupus erythematosus (SLE), and type III IFNs (IFN-λ) were recently also associated with SLE. In this study, we measured levels of IFN-α, IFN-λ1, and related cytokines, such as IL-17A, IL-23, and interferon-γ-induced protein 10 (IP-10), in a Karolinska University Hospital cohort of patients with SLE and control subjects. The objective of the study was to investigate if cytokine measurements could identify different subsets of patients with active SLE and higher disease damage...
June 15, 2017: Arthritis Research & Therapy
https://www.readbyqxmd.com/read/28617419/utilization-of-genomic-sequencing-for-population-screening-of-immunodeficiencies-in-the-newborn
#8
Ashleigh R Pavey, Dale L Bodian, Thierry Vilboux, Alina Khromykh, Natalie S Hauser, Kathi Huddleston, Elisabeth Klein, Aaron Black, Megan S Kane, Ramaswamy K Iyer, John E Niederhuber, Benjamin D Solomon
PurposeImmunodeficiency screening has been added to many state-directed newborn screening programs. The current methodology is limited to screening for severe T-cell lymphopenia disorders. We evaluated the potential of genomic sequencing to augment current newborn screening for immunodeficiency, including identification of non-T cell disorders.MethodsWe analyzed whole-genome sequencing (WGS) and clinical data from a cohort of 1,349 newborn-parent trios by genotype-first and phenotype-first approaches. For the genotype-first approach, we analyzed predicted protein-impacting variants in 329 immunodeficiency-related genes in the WGS data...
June 15, 2017: Genetics in Medicine: Official Journal of the American College of Medical Genetics
https://www.readbyqxmd.com/read/28607757/lymphocyte-subtypes-in-relapsing-remitting-multiple-sclerosis-patients-treated-with-dimethyl-fumarate
#9
C Chaves, R Ganguly, C Ceresia, A Camac
BACKGROUND: Recent data suggest that lymphopenia is more prevalent than reported in relapsing-remitting multiple sclerosis (RRMS) patients taking dimethyl fumarate (DMF). OBJECTIVE: The objective of this study was to investigate the effect of DMF on lymphocyte subtypes in RRMS patients with and without lymphopenia. METHOD: A retrospective study compared lymphocyte subtypes in DMF-treated RRMS patients with low (G1, n = 35) and normal lymphocyte counts (G2, n = 24)...
April 2017: Multiple Sclerosis Journal—Experimental, Translational and Clinical
https://www.readbyqxmd.com/read/28605448/efficacy-of-peptide-receptor-radionuclide-therapy-prrt-for-functional-metastatic-paraganglioma-and-phaeochromocytoma
#10
Grace Kong, Simona Grozinsky-Glasberg, Michael S Hofman, Jason Callahan, Amichay Meirovitz, Ofra Maimon, David A Pattison, David J Gross, Rodney J Hicks
Purpose: Treatment options for unresectable Paraganglioma (PGL)/Phaeochromocytoma (PCC), especially with uncontrolled secondary hypertension (HTN) are limited. Preliminary studies with PRRT suggest efficacy but data on HTN control and survival are lacking. We assessed PRRT outcomes in such patients from 2 referral centres. Methods: 20 consecutive patients (M:F=13:7; 21-77y.o.) with high somatostatin receptor (SSTR) expression treated with 177Lu-DOTA-octreotate, 9 with radiosensitising chemotherapy, were retrospectively reviewed...
June 9, 2017: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28603521/novel-combined-immune-deficiency-and-radiation-sensitivity-blended-phenotype-in-an-adult-with-biallelic-variations-in-zap70-and-rnf168
#11
Ivan K Chinn, Robert P Sanders, Asbjørg Stray-Pedersen, Zeynep H Coban-Akdemir, Vy Hong-Diep Kim, Harjit Dadi, Chaim M Roifman, Troy Quigg, James R Lupski, Jordan S Orange, I Celine Hanson
With the advent of high-throughput genomic sequencing techniques, novel genetic etiologies are being uncovered for previously unexplained Mendelian phenotypes, and the underlying genetic architecture of disease is being unraveled. Although most of these "mendelizing" disease traits represent phenotypes caused by single-gene defects, a percentage of patients have blended phenotypes caused by pathogenic variants in multiple genes. We describe an adult patient with susceptibility to bacterial, herpesviral, and fungal infections...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28590841/rapid-prediction-of-hematologic-acute-radiation-syndrome-in-radiation-injury-patients-using-peripheral-blood-cell-counts
#12
M Port, B Pieper, T Knie, H Dörr, A Ganser, D Graessle, V Meineke, M Abend
Rapid clinical triage of radiation injury patients is essential for determining appropriate diagnostic and therapeutic interventions. We examined the utility of blood cell counts (BCCs) in the first three days postirradiation to predict clinical outcome, specifically for hematologic acute radiation syndrome (HARS). We analyzed BCC test samples from radiation accident victims (n = 135) along with their clinical outcome HARS severity scores (H1-4) using the System for Evaluation and Archiving of Radiation Accidents based on Case Histories (SEARCH) database...
June 7, 2017: Radiation Research
https://www.readbyqxmd.com/read/28581691/recombinant-factor-xiii-prophylaxis-is-safe-and-effective-in-young-children-with-congenital-fxiii-a-deficiency-international-phase-3b-trial-results
#13
Bryce A Kerlin, Aida Inbal, Andrew Will, Michael Williams, May-Lill Garly, Lotte Jacobsen, Susan L Kearney
BACKGROUND: Factor XIII deficiency is a rare, severe congenital bleeding disorder. Monthly prophylaxis with recombinant factor XIII (rFXIII) has demonstrated favorable safety and efficacy in patients ≥6 years and may similarly benefit younger children. OBJECTIVE: To evaluate the long-term safety and efficacy of rFXIII in children <6 years with congenital FXIII A-subunit deficiency. PATIENTS/METHODS: Six children, who previously completed a single-dose pharmacokinetic trial of rFXIII, received 35 IU kg(-1) rFXIII every 28 (± 2) days for a minimum of 52 weeks and were evaluated for bleeding and adverse events...
June 5, 2017: Journal of Thrombosis and Haemostasis: JTH
https://www.readbyqxmd.com/read/28579139/a-phase-i-study-of-neoadjuvant-chemotherapy-with-nab-paclitaxel-doxorubicin-and-cyclophosphamide-in-patients-with-stage-ii-to-iii-breast-cancer
#14
Theresa L Werner, Abhijit Ray, John G Lamb, Matthew VanBrocklin, Kristin Hueftle, Adam L Cohen, Anna C Beck, Saundra S Buys, Donna L Dyess, Thomas W Butler, Theresa L Dumlao, Leigh Neumayer, Hung T Khong
BACKGROUND: The aims of this study were to assess the safety and tolerability of nanoparticle albumin bound paclitaxel (nab-paclitaxel), doxorubicin, and cyclophosphamide as combination therapy for breast cancer patients in the neoadjuvant setting and to assess the overall clinical response and pathologic complete response (pCR). PATIENTS AND METHODS: Twenty-six women with newly diagnosed stage II to III histologically or cytologically proven adenocarcinoma of the breast with negative HER2 status were enrolled...
April 29, 2017: Clinical Breast Cancer
https://www.readbyqxmd.com/read/28576902/application-of-pharmacogenomics-to-investigate-adverse-drug-reactions-to-the-disease-modifying-treatments-for-multiple-sclerosis-a-case-control-study-protocol-for-dimethyl-fumarate-induced-lymphopenia
#15
Kaarina Kowalec, Elaine Kingwell, Robert Carruthers, Ruth Ann Marrie, Sasha Bernatsky, Anthony Traboulsee, Colin J D Ross, Bruce Carleton, Helen Tremlett
INTRODUCTION: Adverse drug reactions (ADRs) are a global public health issue. The potential for pharmacogenomic biomarkers has been demonstrated in several therapeutical areas, including HIV infection and oncology. Dimethyl fumarate (DMF) is a licensed disease-modifying therapy for the treatment of multiple sclerosis (MS). The use of DMF in MS has been associated with a severe reduction in lymphocyte counts and reports of progressive multifocal leukoencephalopathy. Here, we outline the protocol for a case-control study designed to discover genomic variants associated with DMF-induced lymphopenia...
June 2, 2017: BMJ Open
https://www.readbyqxmd.com/read/28575178/predominance-of-cd4-t-cells-in-t-cell-histiocyte-rich-large-b-cell-lymphoma-and-identification-of-a-subset-of-patients-with-peripheral-b-cell-lymphopenia
#16
Christian Kunder, Michael J Cascio, Anthony Bakke, Girish Venkataraman, Dennis P O'Malley, Robert S Ohgami
Objectives: T-cell/histiocyte-rich large B-cell lymphoma (THRLBCL) is a morphologic variant of large B-cell lymphoma whose flow cytometry findings are not well characterized. Methods: Nineteen cases with flow cytometric immunophenotyping were identified from the case records of four institutions between 2001 and 2016. Results: In most cases, neoplastic B cells were not detected by flow cytometry. Overall, cases showed a predominance of CD4+ T cells, which in some cases was marked...
June 1, 2017: American Journal of Clinical Pathology
https://www.readbyqxmd.com/read/28571224/waldmann-s-disease-primary-intestinal-lymphangiectasia-with-atrial-septal-defect
#17
Shrikiran Aroor, Suneel Mundkur, Shravan Kanaparthi, Sandeep Kumar
Waldmann's disease or Primary Intestinal Lymphangiectasia (PIL) is a rare disorder of gastrointestinal tract characterized by dilated lymphatics and widened villi causing leakage of lymph into intestinal lumen. Loss of lymph leads to hypoalbuminemia, hyogammaglobulinemia and lymphopenia. Secondary lymphangiectasia occurs secondary to an elevated lymphatic pressure as in lymphoma, systemic lupus erythematosus, constrictive pericarditis, cardiac surgeries (Fontan's procedure), inflammatory bowel disease and malignancies...
April 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28558844/radiation-related-lymphopenia-is-associated-with-spleen-irradiation-dose-during-radiotherapy-in-patients-with-hepatocellular-carcinoma
#18
Jing Liu, Qianqian Zhao, Weiye Deng, Jie Lu, Xiaoqing Xu, Renben Wang, Xia Li, Jinbo Yue
BACKGROUND: The decrease in peripheral blood lymphocytes induced by radiation lessens the antitumour effect of the immune response, which might cause immunosuppression. We aimed to investigate the correlation between the decrease in peripheral blood lymphocytes during radiotherapy (RT) and the spleen irradiation dose in patients with hepatocellular carcinoma (HCC). METHODS: The subjects were 59 patients with HCC who had received RT from 2005 to 2014. The Min ALC (minimum value of absolute counts for peripheral blood lymphocytes) was collected from the routine workup for each patient prior to RT and weekly during RT...
May 30, 2017: Radiation Oncology
https://www.readbyqxmd.com/read/28555258/peripheral-blood-t-cell-alterations-in-newly-diagnosed-diffuse-large-b-cell-lymphoma-patients-and-their-long-term-dynamics-upon-rituximab-based-chemoimmunotherapy
#19
Simone Battella, M Christina Cox, Raffaella La Scaleia, Arianna Di Napoli, Francesca Di Landro, Alessandra Porzia, Lavinia Franchitti, Fabrizio Mainiero, Luigi Ruco, Bruno Monarca, Angela Santoni, Gabriella Palmieri
The importance of T cell-dependent immune responses in achieving long-term cure of chemoimmunotherapy-treated cancer patients is underscored by the recently described "vaccinal effect" exerted by therapeutic mAbs. In accordance, pre- and post-therapy peripheral blood lymphopenia represents a well-established negative prognostic factor in DLBCL. We analyzed the phenotypic and functional (IFNγ production, and Granzyme B (GrzB) cytotoxic granule marker expression) profile of peripheral blood T lymphocyte subsets ("conventional" CD4(+) and CD8(+), FOXP3(+)CD25(bright) Treg, and "innate-like" CD56(+)) in DLBCL patients at diagnosis, and assessed the long-term impact of R-CHOP chemoimmunotherapy, in a prospective study...
May 29, 2017: Cancer Immunology, Immunotherapy: CII
https://www.readbyqxmd.com/read/28550161/lymphoid-differentiation-of-hematopoietic-stem-cells-requires-efficient-cxcr4-desensitization
#20
Christelle Freitas, Monika Wittner, Julie Nguyen, Vincent Rondeau, Vincent Biajoux, Marie-Laure Aknin, Françoise Gaudin, Sarah Beaussant-Cohen, Yves Bertrand, Christine Bellanné-Chantelot, Jean Donadieu, Françoise Bachelerie, Marion Espéli, Ali Dalloul, Fawzia Louache, Karl Balabanian
The CXCL12/CXCR4 signaling exerts a dominant role in promoting hematopoietic stem and progenitor cell (HSPC) retention and quiescence in bone marrow. Gain-of-function CXCR4 mutations that affect homologous desensitization of the receptor have been reported in the WHIM Syndrome (WS), a rare immunodeficiency characterized by lymphopenia. The mechanisms underpinning this remain obscure. Using a mouse model with a naturally occurring WS-linked gain-of-function Cxcr4 mutation, we explored the possibility that the lymphopenia in WS arises from defects at the HSPC level...
May 26, 2017: Journal of Experimental Medicine
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