1,750 results match your criteria Combined B-Cell and T-Cell Disorders


Progressive Immunodeficiency with Gradual Depletion of B and CD4⁺ T Cells in Immunodeficiency, Centromeric Instability and Facial Anomalies Syndrome 2 (ICF2).

Diseases 2019 Apr 4;7(2). Epub 2019 Apr 4.

Department of Clinical Immunology and Rheumatology, Hannover Medical School, 30625 Hannover, Germany.

Immunodeficiency, centromeric instability and facial anomalies syndrome 2 (ICF2) is a rare autosomal recessive primary immunodeficiency disorder. So far, 27 patients have been reported. Here, we present three siblings with ICF2 due to a homozygous ZBTB24 gene mutation (c. Read More

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http://dx.doi.org/10.3390/diseases7020034DOI Listing
April 2019
1 Read

An ODE-based mixed modelling approach for B- and T-cell dynamics induced by Varicella-Zoster Virus vaccines in adults shows higher T-cell proliferation with Shingrix than with Varilrix.

Vaccine 2019 May 8;37(19):2537-2553. Epub 2019 Apr 8.

Centre for Health Economics Research & Modeling Infectious Diseases (CHERMID), Vaccine & Infectious Disease Institute (VAXINFECTIO), University of Antwerp, Antwerp, Belgium; Antwerp Unit for Data Analysis and Computation in Immunology and Sequencing (AUDACIS), University of Antwerp, Antwerp, Belgium; Interuniversity Institute for Biostatistics and statistical Bioinformatics, Hasselt University, Diepenbeek, Belgium.

Clinical trials covering the immunogenicity of a vaccine aim to study the longitudinal dynamics of certain immune cells after vaccination. The corresponding immunogenicity datasets are mainly analyzed by the use of statistical (mixed effects) models. This paper proposes the use of mathematical ordinary differential equation (ODE) models, combined with a mixed effects approach. Read More

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http://dx.doi.org/10.1016/j.vaccine.2019.03.075DOI Listing
May 2019
1 Read

Skewed B cell receptor repertoire and reduced antibody avidity in patients with DOCK8 deficiency.

Scand J Immunol 2019 Feb 21:e12759. Epub 2019 Feb 21.

Division of Immunology, Children's Hospital of Chongqing Medical University, Chongqing, China.

DOCK8 immunodeficiency syndrome (DIDS) is a combined immunodeficiency characterized by recurrent viral infections, severe atopy and early onset malignancy. Immunological abnormalities include lymphopenia, CD8 T-cell cytoskeleton dysfunction, defective B cell memory and variable serum immunoglobulin levels. Here, we analyse the B cell receptor repertoire (BCR) characteristics and antibody avidity of four DIDS patients, attempt to understand the dysregulated humoral immunity in DIDS patients with a normal antibody titre and suggest a scientific basis for intravenous immunoglobulin (IVIG) replacement therapy for these patients. Read More

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http://dx.doi.org/10.1111/sji.12759DOI Listing
February 2019

Atypical SIFD with novel TRNT1 mutations: a case study on the pathogenesis of B-cell deficiency.

Int J Hematol 2019 Apr 13;109(4):382-389. Epub 2019 Feb 13.

Department of Pediatrics and Developmental Biology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University (TMDU), 1-5-45 Yushima, Bunkyo-ku, Tokyo, 113-8510, Japan.

Mutation in the gene encoding tRNA nucleotidyl transferase, CCA-adding 1 (TRNT1), an enzyme essential for the synthesis of the 3'-terminal CCA sequence in tRNA molecules, results in a disorder that features sideroblastic anemia, B-cell immunodeficiency, periodic fever, and developmental delay. Mutations in TRNT1 are also linked to phenotypes including retinitis pigmentosa, cataracts, and cardiomyopathy. To date, it has remained unclear how defective TRNT1 is linked to B-cell deficiency. Read More

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http://link.springer.com/10.1007/s12185-019-02614-0
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http://dx.doi.org/10.1007/s12185-019-02614-0DOI Listing
April 2019
11 Reads

Pediatric non-Hodgkin lymphoma: Characteristics, stratification, and treatment at a single institute in Thailand.

Pediatr Int 2019 Jan;61(1):49-57

Department of Pediatrics, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.

Background: In the modern era of chemotherapy, the outcome of pediatric non-Hodgkin lymphoma (NHL) continues to improve internationally. Limited data such as information on epidemiology and survival, however, are available in Asian countries.

Methods: Children (≤15 years old) diagnosed with histologically proven NHL from 1998 to 2014 were retrospectively analyzed. Read More

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http://dx.doi.org/10.1111/ped.13739DOI Listing
January 2019
2 Reads

[Primary skin lymphomas: Current therapy].

Authors:
O Dereure

Ann Dermatol Venereol 2019 Feb 30;146(2):92-99. Epub 2019 Jan 30.

Unité Inserm U1058, département de dermatologie, université Montpellier, hôpital Saint-Éloi, 80, avenue Augustin-Fliche, 34295 Montpellier cedex 5, France. Electronic address:

Therapeutic progress in primary cutaneous lymphomas continues to be largely dominated by the T-cell lymphomas, towards which the great majority of recent therapeutic innovations have been directed. The latter include local treatments consisting either of relatively classical but "revamped" approaches involving different pharmaceutical forms (example: chlormethine gel) or else lower but seemingly equally effective dosages (electron therapy), or of more innovative approaches (example: UVA-1, dynamic phototherapy, imiquimod, resimiquimod). However, significant progress has been made chiefly in terms of systemic treatments with the emergence of "targeted" drugs that directly and specifically target tumour cells (monoclonal antibodies directed against CD30, CCR4 or CD158k) and the further development of "small" molecules such as histone deacetylase inhibitors and new cytostatics. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S01519638183135
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http://dx.doi.org/10.1016/j.annder.2018.11.012DOI Listing
February 2019
8 Reads

Aging and End Stage Renal Disease Cause A Decrease in Absolute Circulating Lymphocyte Counts with A Shift to A Memory Profile and Diverge in Treg Population.

Aging Dis 2019 Feb 1;10(1):49-61. Epub 2019 Feb 1.

2Renal Transplant Service, Hospital das Clinicas, University of Sao Paulo School of Medicine, Sao Paulo, Brazil.

There is a growing number of elderly kidney transplant (Ktx) recipients. Elderly recipients present lower acute rejection rates but higher incidence of infection and malignancies. Aging seems to result in a shift to memory profile and chronic kidney disease (CKD) in premature immunological aging. Read More

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http://dx.doi.org/10.14336/AD.2018.0318DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6345336PMC
February 2019
2 Reads

A proliferation-inducing ligand-mediated anti-inflammatory response of astrocytes in multiple sclerosis.

Ann Neurol 2019 Mar 4;85(3):406-420. Epub 2019 Feb 4.

Institute for Advanced Biosciences, Grenoble Alpes University/National Institute of Health and Medical Research U1209/National Center for Scientific Research UMR5309, La Tronche, France.

Objective: The two related tumor necrosis factor members a proliferation-inducing ligand (APRIL) and B-cell activation factor (BAFF) are currently targeted in autoimmune diseases as B-cell regulators. In multiple sclerosis (MS), combined APRIL/BAFF blockade led to unexpected exacerbated inflammation in the central nervous system (CNS) of patients. Here, we investigate the role of the APRIL/BAFF axis in the CNS. Read More

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http://dx.doi.org/10.1002/ana.25415DOI Listing
March 2019
2 Reads

Human RIPK1 deficiency causes combined immunodeficiency and inflammatory bowel diseases.

Proc Natl Acad Sci U S A 2019 01 27;116(3):970-975. Epub 2018 Dec 27.

Dr. von Hauner Children's Hospital, Department of Pediatrics, University Hospital, Ludwig-Maximilians-Universität (LMU) Munich, 80337 Munich, Germany;

Receptor-interacting serine/threonine-protein kinase 1 (RIPK1) is a critical regulator of cell death and inflammation, but its relevance for human disease pathogenesis remains elusive. Studies of monogenic disorders might provide critical insights into disease mechanisms and therapeutic targeting of RIPK1 for common diseases. Here, we report on eight patients from six unrelated pedigrees with biallelic loss-of-function mutations in presenting with primary immunodeficiency and/or intestinal inflammation. Read More

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http://dx.doi.org/10.1073/pnas.1813582116DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6338855PMC
January 2019
23 Reads

Adaptive Immunodeficiency in WHIM Syndrome.

Int J Mol Sci 2018 Dec 20;20(1). Epub 2018 Dec 20.

Molecular Signaling Section, Laboratory of Molecular Immunology, National Institute of Allergy and Infectious Diseases, NIH, Bethesda, MD 20892, USA.

Cysteine-X-cysteine chemokine receptor 4 (CXCR4) is a broadly expressed and multifunctional G protein-coupled chemokine receptor critical for organogenesis, hematopoiesis, and antimicrobial host defense. In the hematopoietic system, the binding of CXCR4 to its cognate chemokine ligand, CXCL12, mediates leukocyte trafficking, distribution, survival, activation, and proliferation. Warts, hypogammaglobulinemia, infections, and myelokathexis (WHIM) syndrome is a rare, autosomal dominant, combined immunodeficiency disorder caused by mutations in the -terminus of CXCR4 that prevent receptor downregulation and therefore result in pathologically increased signaling. Read More

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http://www.mdpi.com/1422-0067/20/1/3
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http://dx.doi.org/10.3390/ijms20010003DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6337672PMC
December 2018
19 Reads

Sex differences of inflammation in target organs, induced by intraperitoneal injection of lipopolysaccharide, depend on its dose.

J Inflamm Res 2018 8;11:431-445. Epub 2018 Nov 8.

Department of Pathology, Federal State Budgetary Institution "Science Research Institute of Human Morphology", Moscow, Russia.

Purpose: The aim of our research was to study sex differences and the severity of inflammatory changes in target organs and the peculiarities of immunological disorders when low and high doses of lipopolysaccharide (LPS) were administered to rats.

Methods: Male and female 2- to 3-month-old Wistar rats (200-250 g) were injected intraperitoneally with LPS in one of two doses: 1.5 or 15 mg/kg. Read More

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https://www.dovepress.com/sex-differences-of-inflammation-in
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http://dx.doi.org/10.2147/JIR.S178288DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6233486PMC
November 2018
24 Reads

Loss of human ICOSL results in combined immunodeficiency.

J Exp Med 2018 Dec;215(12):3151-3164

Infectious Disease Susceptibility Program, McGill University Health Centre and Research Institute-McGill University Health Centre, Montréal, Québec, Canada

Primary immunodeficiencies represent naturally occurring experimental models to decipher human immunobiology. We report a patient with combined immunodeficiency, marked by recurrent respiratory tract and DNA-based viral infections, hypogammaglobulinemia, and panlymphopenia. He also developed moderate neutropenia but without prototypical pyogenic infections. Read More

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http://dx.doi.org/10.1084/jem.20180668DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6279397PMC
December 2018
18 Reads

Efficacy of the GMALL-B-ALL/NHL2002 protocol in Burkitt leukemia/lymphoma and aggressive non-Hodgkin-lymphomas with or without CNS involvement.

Eur J Haematol 2019 Mar 20;102(3):241-250. Epub 2019 Jan 20.

Department of Haematology, University Hospital Essen, University of Duisburg-Essen, Essen, Germany.

Objectives: The GMALL-B-ALL/NHL2002 protocol is effective in Burkitt lymphoma/leukemia (BL). Its role in other aggressive lymphomas and in patients with simultaneous central nervous system (CNS) and peripheral involvement is unclear.

Methods: This is a retrospective outcome analysis in 76 patients with BL (n = 26), B-lymphoblastic lymphoma (B-LBL; n = 3), diffuse large B-cell lymphoma (DLBCL; n = 31), mantle cell lymphoma (MCL; n = 6), transformed B-cell non-Hodgkin lymphomas (tB-NHL; n = 7), and T-cell NHL (T-NHL; n = 3) treated with the GMALL-B-ALL/NHL2002 protocol. Read More

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http://dx.doi.org/10.1111/ejh.13199DOI Listing
March 2019
38 Reads

Should immunologic strategies be incorporated into frontline ALL therapy?

Best Pract Res Clin Haematol 2018 12 25;31(4):367-372. Epub 2018 Sep 25.

Pediatric Oncology Research Laboratory and Department of Hematology, Rigshospitalet, University of Copenhagen, Copenhagen, Denmark; Department of Medicine, University of Chicago Medicine, Knapp Center for Biomedical Discovery, 900 E. 57th Street, 8th Floor Chicago, IL 60637, USA. Electronic address:

Survival rates in adult patients with acute lymphoblastic leukemia (ALL) have markedly improved during the past decade. The one-size-fits-all-ages approach has been replaced with adaptation of pediatric-inspired treatment protocols for younger adults. Yet different treatment strategies for older patients are needed due to chemotherapy-related toxicities. Read More

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http://dx.doi.org/10.1016/j.beha.2018.09.015DOI Listing
December 2018
21 Reads

CAR-T bridging to allo-HSCT as a treatment strategy for relapsed adult acute B-lymphoblastic leukemia: a case report.

BMC Cancer 2018 Nov 20;18(1):1143. Epub 2018 Nov 20.

Department of Hematology, The Second Hospital of Hebei Medical University, Hebei Key Laboratory of Hematology, Shijiazhuang, 050000, Hebei, China.

Background: Adults with relapsed acute lymphoblastic leukemia (ALL) have a poor prognosis, especially in patients who relapsed within 6 months of complete remission 1 (CR1). Allogeneic hematopoietic stem cell transplantation (allo-HSCT) is the treatment of choice. However, this can only be considered after complete remission 2 (CR2) is achieved. Read More

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http://dx.doi.org/10.1186/s12885-018-5037-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6245806PMC
November 2018
13 Reads

Role of humoral immunity against hepatitis B virus core antigen in the pathogenesis of acute liver failure.

Proc Natl Acad Sci U S A 2018 11 12;115(48):E11369-E11378. Epub 2018 Nov 12.

Hepatic Pathogenesis Section, Laboratory of Infectious Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD 20892;

Hepatitis B virus (HBV)-associated acute liver failure (ALF) is a dramatic clinical syndrome leading to death or liver transplantation in 80% of cases. Due to the extremely rapid clinical course, the difficulties in obtaining liver specimens, and the lack of an animal model, the pathogenesis of ALF remains largely unknown. Here, we performed a comprehensive genetic and functional characterization of the virus and the host in liver tissue from HBV-associated ALF and compared the results with those of classic acute hepatitis B in chimpanzees. Read More

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http://www.pnas.org/lookup/doi/10.1073/pnas.1809028115
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http://dx.doi.org/10.1073/pnas.1809028115DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6275524PMC
November 2018
15 Reads

Peptide vaccine against chikungunya virus: immuno-informatics combined with molecular docking approach.

J Transl Med 2018 10 27;16(1):298. Epub 2018 Oct 27.

Hubei Key Laboratory of Agricultural Bioinformatics, College of Informatics, Huazhong Agricultural University (HZAU), Wuhan, People's Republic of China.

Background: Chikungunya virus (CHIKV), causes massive outbreaks of chikungunya infection in several regions of Asia, Africa and Central/South America. Being positive sense RNA virus, CHIKV replication within the host resulting in its genome mutation and led to difficulties in creation of vaccine, drugs and treatment strategies. Vector control strategy has been a gold standard to combat spreading of CHIKV infection, but to eradicate a species from the face of earth is not an easy task. Read More

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https://translational-medicine.biomedcentral.com/articles/10
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http://dx.doi.org/10.1186/s12967-018-1672-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6204282PMC
October 2018
17 Reads
3.930 Impact Factor

CD3/CD19 Depleted Matched and Mismatched Unrelated Donor Hematopoietic Stem Cell Transplant with Targeted T Cell Addback Is Associated with Excellent Outcomes in Pediatric Patients with Nonmalignant Hematologic Disorders.

Biol Blood Marrow Transplant 2019 Mar 9;25(3):549-555. Epub 2018 Oct 9.

Cellular Therapy and Transplant Section, Division of Oncology Children's Hospital of Philadelphia, Philadelphia, Pennsylvania; Department of Pediatrics, Perelman School of Medicine of the University of Pennsylvania, Philadelphia, Pennsylvania. Electronic address:

Unrelated donor hematopoietic stem cell transplantation (HSCT) is increasingly being used to cure nonmalignant hematologic diseases (NMHD) in patients who lack HLA matched related donors. Both graft rejection and graft-versus-host disease (GVHD) remain major barriers to safe and effective transplant for these patients requiring unrelated donors. Partial T cell depletion combined with peripheral stem cell transplantation (pTCD-PSCT) has the potential advantages of providing a high stem cell dose to facilitate rapid engraftment, maintaining cells that may facilitate engraftment, and decreasing GVHD risk compared with T cell-replete HSCT. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S10838791183061
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http://dx.doi.org/10.1016/j.bbmt.2018.10.003DOI Listing
March 2019
10 Reads

The Case for Cardiac Xenotransplantation in Neonates: Is Now the Time to Reconsider Xenotransplantation for Hypoplastic Left Heart Syndrome?

Pediatr Cardiol 2019 Feb 9;40(2):437-444. Epub 2018 Oct 9.

Xenotransplantation Program, Department of Surgery, University of Alabama at Birmingham, Birmingham, AL, USA.

Neonatal cardiac transplantation for hypoplastic left heart syndrome (HLHS) is associated with excellent long-term survival compared to older recipients. However, heart transplantation for neonates is greatly limited by the critical shortage of donor hearts, and by the associated mortality of the long pre-transplant waiting period. This led to the development of staged surgical palliation as the first-line surgical therapy for HLHS. Read More

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http://link.springer.com/10.1007/s00246-018-1998-1
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http://dx.doi.org/10.1007/s00246-018-1998-1DOI Listing
February 2019
15 Reads

Primary immunodeficiency.

Allergy Asthma Clin Immunol 2018 12;14(Suppl 2):61. Epub 2018 Sep 12.

4University of Manitoba, Winnipeg, MB Canada.

Primary immunodeficiency disorder (PID) refers to a large heterogeneous group of disorders that result from defects in immune system development and/or function. PIDs are broadly classified as disorders of adaptive immunity (i.e. Read More

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http://dx.doi.org/10.1186/s13223-018-0290-5DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6157160PMC
September 2018
3 Reads

T-cell defects in patients with germline mutations account for combined immunodeficiency.

Blood 2018 Nov 25;132(22):2362-2374. Epub 2018 Sep 25.

San Raffaele Telethon Institute for Gene Therapy, Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS) San Raffaele Scientific Institute, Milan, Italy.

ARPC1B is a key factor for the assembly and maintenance of the ARP2/3 complex that is involved in actin branching from an existing filament. Germline biallelic mutations in have been recently described in 6 patients with clinical features of combined immunodeficiency (CID), whose neutrophils and platelets but not T lymphocytes were studied. We hypothesized that ARPC1B deficiency may also lead to cytoskeleton and functional defects in T cells. Read More

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http://www.bloodjournal.org/lookup/doi/10.1182/blood-2018-07
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http://dx.doi.org/10.1182/blood-2018-07-863431DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6265646PMC
November 2018
32 Reads

IL-7R-mediated signaling in T-cell acute lymphoblastic leukemia: An update.

Adv Biol Regul 2019 Jan 19;71:88-96. Epub 2018 Sep 19.

Instituto de Medicina Molecular João Lobo Antunes, Faculdade de Medicina, Universidade de Lisboa, Av. Prof. Egas Moniz, 1649-028, Lisboa, Portugal. Electronic address:

Interleukin 7 (IL-7) and its receptor (IL-7R, a heterodimer of IL-7Rα and γc) are essential for normal lymphoid development. In their absence, severe combined immunodeficiency occurs. By contrast, excessive IL-7/IL-7R-mediated signaling can drive lymphoid leukemia development, disease acceleration and resistance to chemotherapy. Read More

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http://dx.doi.org/10.1016/j.jbior.2018.09.012DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6386770PMC
January 2019
22 Reads

Venetoclax in Combination with Decitabine for Relapsed T-Cell Acute Lymphoblastic Leukemia after Allogeneic Hematopoietic Cell Transplant.

Case Rep Hematol 2018 26;2018:6092646. Epub 2018 Aug 26.

Department of Hematologic Malignancies and Blood and Marrow Transplant, University of California, San Francisco, CA, USA.

Long-term disease-free survival in adults with T-cell acute lymphoblastic leukemia (T-ALL) remains poor, particularly after relapse, with few available salvage options. Preclinical data suggest that inhibition of the antiapoptotic protein BCL-2 (B-cell lymphoma 2) either alone or in combination with other agents, may be a unique therapeutic approach for the treatment of T-ALL. We present a case of a young male with T-ALL, relapsed after allogeneic hematopoietic stem cell transplant, who achieved a second complete remission following salvage therapy with combined venetoclax and decitabine. Read More

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http://dx.doi.org/10.1155/2018/6092646DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6129347PMC
August 2018
4 Reads

The prognostic value of programmed cell death ligand 1 expression in non-Hodgkin lymphoma: a meta-analysis.

Cancer Biol Med 2018 Aug;15(3):290-298

Department of Medical Oncology.

Objective: Although the prognostic value of programmed cell death-ligand 1 (PD-L1) expression in non-Hodgkin lymphoma (NHL) has been evaluated in many studies, the results remain controversial. To investigate the prognostic role of PD-L1 expression and the association between PD-L1 expression and clinicopathological features of NHL, we performed a meta-analysis.

Methods: The PubMed, EMBASE, and Cochrane Library databases were searched up to November 30, 2017. Read More

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http://dx.doi.org/10.20892/j.issn.2095-3941.2018.0047DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6121049PMC
August 2018
3 Reads

Synergistic antitumor effect of suberoylanilide hydroxamic acid and cisplatin in osteosarcoma cells.

Oncol Lett 2018 Oct 27;16(4):4663-4670. Epub 2018 Jul 27.

Key Laboratory of Diagnostic Medicine Designated by The Chinese Ministry of Education, Department of Laboratory Medicine, Chongqing Medical University, Chongqing 400016, P.R. China.

Cisplatin, as a first-line chemotherapy drug, has been widely applied for therapy of osteosarcoma. However, its application is limited by drug resistance and serious side effects, including nephrotoxicity and ototoxicity. Suberoylanilide hydroxamic acid (SAHA) is a newly developed histone deacetylase (HDAC) inhibitor, which is the first Food and Drug Administration-approved HDAC inhibitor for the treatment of cutaneous manifestations of T-cell lymphoma. Read More

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http://www.spandidos-publications.com/10.3892/ol.2018.9224
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http://dx.doi.org/10.3892/ol.2018.9224DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6126343PMC
October 2018
22 Reads

Effective suppression of donor specific antibody production by Cathepsin S inhibitors in a mouse transplantation model.

Eur J Pharmacol 2018 Nov 6;838:145-152. Epub 2018 Sep 6.

Drug Discovery Research, Astellas Pharma Inc., 21, Miyukigaoka, Tsukuba-shi, Ibaraki 305-8585, Japan.

Donor-specific antibodies (DSA) are a major risk factor for antibody-mediated rejection (ABMR) in solid organ transplantation, and ABMR remains a medical challenge. Therefore, effective anti-ABMR therapies are needed to improve overall graft survival. Cathepsin S (Cat S) is an essential protease for antigen peptide loading onto lysosomal/endosomal major histocompatibility complex (MHC) class II molecules to promote antigen presentation. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S00142999183052
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http://dx.doi.org/10.1016/j.ejphar.2018.09.007DOI Listing
November 2018
18 Reads

Hypomorphic caspase activation and recruitment domain 11 (CARD11) mutations associated with diverse immunologic phenotypes with or without atopic disease.

J Allergy Clin Immunol 2019 Apr 28;143(4):1482-1495. Epub 2018 Aug 28.

Department of Pharmacology & Molecular Therapeutics, Uniformed Services University of the Health Sciences, Bethesda, Md. Electronic address:

Background: Caspase activation and recruitment domain 11 (CARD11) encodes a scaffold protein in lymphocytes that links antigen receptor engagement with downstream signaling to nuclear factor κB, c-Jun N-terminal kinase, and mechanistic target of rapamycin complex 1. Germline CARD11 mutations cause several distinct primary immune disorders in human subjects, including severe combined immune deficiency (biallelic null mutations), B-cell expansion with nuclear factor κB and T-cell anergy (heterozygous, gain-of-function mutations), and severe atopic disease (loss-of-function, heterozygous, dominant interfering mutations), which has focused attention on CARD11 mutations discovered by using whole-exome sequencing.

Objectives: We sought to determine the molecular actions of an extended allelic series of CARD11 and to characterize the expanding range of clinical phenotypes associated with heterozygous CARD11 loss-of-function alleles. Read More

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http://dx.doi.org/10.1016/j.jaci.2018.08.013DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6395549PMC
April 2019
19 Reads
1 Citation
11.480 Impact Factor

SCID genotype and 6-month posttransplant CD4 count predict survival and immune recovery.

Blood 2018 Oct 28;132(17):1737-1749. Epub 2018 Aug 28.

Laboratory of Clinical Immunology and Microbiology, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, MD.

The Primary Immune Deficiency Treatment Consortium (PIDTC) performed a retrospective analysis of 662 patients with severe combined immunodeficiency (SCID) who received a hematopoietic cell transplantation (HCT) as first-line treatment between 1982 and 2012 in 33 North American institutions. Overall survival was higher after HCT from matched-sibling donors (MSDs). Among recipients of non-MSD HCT, multivariate analysis showed that the SCID genotype strongly influenced survival and immune reconstitution. Read More

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http://dx.doi.org/10.1182/blood-2018-03-840702DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6202916PMC
October 2018
24 Reads

Combined positron emission tomography/computed tomography for diagnosis and monitoring of orbital adnexal lymphoma.

Acta Ophthalmol 2018 Sep 26;96(6):e712-e717. Epub 2018 Aug 26.

Department of Ophthalmology, Ludwig-Maximilians-University, Munich, Germany.

Purpose: Combined whole-body F-18-fluoro-2-deoxyglucose positron emission tomography / computed tomography ([18F]FDG-PET/CT) gives precise information about tumour morphology and metabolism. The standardized uptake value (SUV) allows quantification of tumour metabolism. The diagnostic value of PET/CT in patients with suspected orbital adnexal lymphoma (OAL) was evaluated. Read More

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http://dx.doi.org/10.1111/aos.13766DOI Listing
September 2018
15 Reads

Two siblings with PRKDC defect who presented with cutaneous granulomas and review of the literature.

Clin Immunol 2018 Dec 16;197:1-5. Epub 2018 Aug 16.

Hacettepe University Faculty of Medicine, Department of Pediatrics, Division of Immunology, Ankara, Turkey.

V(D)J recombination, during which recognition and repair of broken DNA chains are accomplished by non-homologous end joining pathway, is a critical process in B and T cell development.Null mutations of each enzyme or protein of this pathway result in T- B- NK+ severe combined immunodeficiency whereas hypomorphic mutations result in atypical(leaky)severe combined immunodeficiency forms. We present two siblings with PRKDC (Protein Kinase, DNA-Activated, Catalytic Polypeptide) mutation who presented with granulomatous skin lesions and recurrent lung infections. Read More

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http://dx.doi.org/10.1016/j.clim.2018.08.002DOI Listing
December 2018
19 Reads

Canine T cell lymphoma treated with lomustine, vincristine, procarbazine, and prednisolone chemotherapy in 35 dogs.

Vet Comp Oncol 2018 Dec 16;16(4):622-629. Epub 2018 Aug 16.

Department of Oncology, Animal Referral Hospital Brisbane, Brisbane, Australia.

Canine T cell lymphoma has previously been found to be a poor prognostic indicator compared with its B cell counterpart. The cyclophosphamide, doxorubicin, vincristine, and prednisolone protocol is widely accepted as a first line treatment for canine lymphoma. There have been several studies investigating alternative protocols for T cell lymphoma. Read More

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http://dx.doi.org/10.1111/vco.12430DOI Listing
December 2018
2 Reads

[De novo NFκB2 gene mutation associated common variable immunodeficiency].

Zhonghua Er Ke Za Zhi 2018 Aug;56(8):628-632

Department of Rheumatology and Immunology, Children's Hospital of Chongqing Medical University, Ministry of Education Key Laboratory of Child Development and Disorders, China International Science and Technology Cooperation Base of Child Development and Critical Disorders, Chongqing Key Laboratory of Child Infection and Immunity, Chongqing 400014, China.

To investigate the clinical, immunological, and molecular manifestations of nuclear factor kappa-B subunit 2 (NFκB2) gene mutation associated common variable immunodeficiency (CVID) . A 14-month-old boy diagnosed with NFκB2-mutated CVID was admitted into Children's Hospital of Chongqing Medical University in December 2015. The clinical manifestations, biochemical tests, immunological function, molecular features, treatment, and follow-up of the patient were analyzed. Read More

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http://dx.doi.org/10.3760/cma.j.issn.0578-1310.2018.08.014DOI Listing
August 2018
4 Reads

A closer look at blinatumomab.

Authors:
Ryan J Mattison

Clin Adv Hematol Oncol 2018 Jun;16(6):412-414

University of Wisconsin School of Medicine and Public Health, Carbone Comprehensive Cancer Center, Madison, Wisconsin.

On March 29, 2018, blinatumomab (Blincyto, Amgen) received an accelerated expanded approval for the treatment of adult and pediatric patients with B-cell precursor acute lymphoblastic leukemia (ALL) who are in first or second complete remission (CR) and have minimal residual disease (MRD). Blinatumomab was first approved for use in adult patients (in December 2014) and later in pediatric patients (in September 2016) with relapsed or refractory Philadelphia chromosome (Ph)-negative B-cell precursor ALL; the approval was expanded in July 2017 to include patients with Ph-positive disease. The agent is a bispecific CD19-directed CD3 T-cell engager. Read More

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June 2018
23 Reads

Lymphoma associated hemophagocytic syndrome: A single-center retrospective study.

Oncol Lett 2018 Jul 22;16(1):1275-1284. Epub 2018 May 22.

Department of Oncology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, Henan 450052, P.R. China.

To improve the understanding of lymphoma associated hemophagocytic syndrome (LAHS) and find an effective treatment for this fatal disease, 57 patients with LAHS were retrospectively reviewed. The most common histopathological type was extranodal natural killer (NK)/T-cell lymphoma, nasal type (ENKL) (45.61%). Read More

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http://dx.doi.org/10.3892/ol.2018.8783DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6063042PMC
July 2018
8 Reads
0.990 Impact Factor

Multiplex ImmunoSpot Assays for the Study of Functional B Cell Subpopulations.

Methods Mol Biol 2018 ;1808:73-83

Cellular Technology Ltd., Shaker Heights, OH, USA.

B cells mediate humoral immunity by producing antibody molecules, but they also participate in innate and acquired immune functions via the secretion of effector molecules such as cytokines, chemokines, and granzyme. B cell subpopulations releasing such effector molecules have been implicated in immunobiology and a number of diseases.Unlike antigen-specific T cells that can be identified by multimer staining, and then counter-stained to define T cell subpopulations, antigen-specific B cells cannot be detected by flow cytometry. Read More

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http://dx.doi.org/10.1007/978-1-4939-8567-8_7DOI Listing

Combined External, Endoscopic, Endonasal-Assisted En Bloc Resection of Malignant Tumors From the Lacrimal Drainage System.

J Craniofac Surg 2018 Oct;29(7):1855-1858

Department of Orbital & Oculoplastic Surgery, Eye Hospital of Wenzhou Medical University, Wenzhou, China.

Purpose: The aim of this study is to describe a minimally invasive technique of en bloc resection of malignant tumors from lacrimal drainage system (LDS).

Methods: This was a noncomparative, retrospective chart review of the clinical and pathologic findings of patients presenting with a LDS malignant tumor who underwent endoscopic prelacrimal recess approach with a small external incision for en bloc excision of the LDS.

Results: A total of 12 patients from April 2010 to July 2017 were reviewed in this study. Read More

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http://dx.doi.org/10.1097/SCS.0000000000004655DOI Listing
October 2018
2 Reads

Newborn screening using TREC/KREC assay for severe T and B cell lymphopenia in Iran.

Scand J Immunol 2018 Jun 26:e12699. Epub 2018 Jun 26.

Immunology, Asthma and Allergy Research Institute, Tehran University of Medical Sciences, Tehran, Iran.

T-cell receptor excision circles (TRECs) and κ-deleting recombination excision circles (KRECs) are recently used for detection of T or B cell lymphopenia in neonates based on region-specific cutoff levels. Here, we report cutoffs for TREC and KREC copies useful for newborn screening and/or diagnosis of primary immunodeficiency diseases (PID) in Iran. DNA was extracted from a single 3. Read More

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http://doi.wiley.com/10.1111/sji.12699
Publisher Site
http://dx.doi.org/10.1111/sji.12699DOI Listing
June 2018
8 Reads

B2-Lymphocyte responses to oxidative stress-derived antigens contribute to the evolution of nonalcoholic fatty liver disease (NAFLD).

Free Radic Biol Med 2018 Aug 18;124:249-259. Epub 2018 Jun 18.

Dept. of Health Sciences, Interdisciplinary Research Centre for Autoimmune Diseases, University "Amedeo Avogadro" of East Piedmont, Novara, Italy. Electronic address:

Recent evidence implicates adaptive immunity as a key player in the mechanisms supporting hepatic inflammation during the progression of nonalcoholic fatty liver disease (NAFLD). In these settings, patients with NAFLD often show an increase in the circulating levels of antibodies against oxidative stress-derived epitopes (OSE). Nonetheless, the actual role of humoral immunity in NAFLD is still unclear. Read More

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http://dx.doi.org/10.1016/j.freeradbiomed.2018.06.015DOI Listing
August 2018
1 Read

Donor-derived CAR-T Cells Serve as a Reduced-intensity Conditioning Regimen for Haploidentical Stem Cell Transplantation in Treatment of Relapsed/Refractory Acute Lymphoblastic Leukemia: Case Report and Review of the Literature.

J Immunother 2018 Jul/Aug;41(6):306-311

Department of Hematology, Xinqiao Hospital.

Background: Reduced-intensity conditioning (RIC) regimens with low tolerable toxicities have been used for allogeneic hematopoietic stem cell transplantation (allo-HSCT). However, the relapse rate by this treatment is high. Treatment of CD19 B-cell relapsed/refractory acute lymphoblastic leukemia (r/r ALL) with allogeneic chimeric antigen receptor-modified T (CAR-T) cells is safe and effective. Read More

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http://dx.doi.org/10.1097/CJI.0000000000000233DOI Listing
June 2018
11 Reads

Survival in HIV-infected patients with lymphoma according to the choice of antiretroviral treatment: an observational multicentre study.

HIV Med 2018 Jun 4. Epub 2018 Jun 4.

Unit of Infectious Diseases, Department of Medical Sciences, University of Torino, Torino, Italy.

Objectives: Lymphoproliferative disorders are often observed in HIV-positive patients. Combination antiretroviral treatment (cART) during antineoplastic chemotherapy is beneficial, but little is known about the clinical outcome according to different antiretroviral combinations. The aim of the study was to address this gap in current knowledge. Read More

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http://dx.doi.org/10.1111/hiv.12624DOI Listing
June 2018
9 Reads

Fludarabine and rituximab with escalating doses of lenalidomide followed by lenalidomide/rituximab maintenance in previously untreated chronic lymphocytic leukaemia (CLL): the REVLIRIT CLL-5 AGMT phase I/II study.

Ann Hematol 2018 Oct 4;97(10):1825-1839. Epub 2018 Jun 4.

Department of Internal Medicine III with Hematology and Medical Oncology, Oncologic Center, Paracelsus Medical University Salzburg, Müllner-Hauptstr. 48, 5020, Salzburg, Austria.

Despite recent advances, chemoimmunotherapy remains a standard for fit previously untreated chronic lymphocytic leukaemia patients. Lenalidomide had activity in early monotherapy trials, but tumour lysis and flare proved major obstacles in its development. We combined lenalidomide in increasing doses with six cycles of fludarabine and rituximab (FR), followed by lenalidomide/rituximab maintenance. Read More

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http://dx.doi.org/10.1007/s00277-018-3380-zDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6097797PMC
October 2018
31 Reads

Gene Expression Profiling in Behcet's Disease Indicates an Autoimmune Component in the Pathogenesis of the Disease and Opens New Avenues for Targeted Therapy.

J Immunol Res 2018 24;2018:4246965. Epub 2018 Apr 24.

Department of Medicine, University of Verona, Piazzale L.A. Scuro 10, 37134 Verona, Italy.

Behçet disease (BD) is a chronic inflammatory multisystem disease characterized by oral and genital ulcers, uveitis, and skin lesions. Disease etiopathogenesis is still unclear. We aim to elucidate some aspects of BD pathogenesis and to identify specific gene signatures in peripheral blood cells (PBCs) of patients with active disease using novel gene expression and network analysis. Read More

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http://dx.doi.org/10.1155/2018/4246965DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5941805PMC
October 2018
12 Reads

Severe combined immunodeficiency in stimulator of interferon genes (STING) V154M/wild-type mice.

J Allergy Clin Immunol 2019 Feb 23;143(2):712-725.e5. Epub 2018 May 23.

CNRS UPR 3572 "Immunopathology and Therapeutic Chemistry"/Laboratory of Excellence Medalis, Institute of Molecular and Cellular Biology (IBMC), Strasbourg, France; Department of Clinical Immunology and Internal Medicine, National Reference Center for Autoimmune Diseases, Hôpitaux Universitaires de Strasbourg, Strasbourg, France; UFR Sciences Pharmaceutiques, Université de Strasbourg, Illkirch-Graffenstaden, France. Electronic address:

Background: Autosomal dominant gain-of-function mutations in human stimulator of interferon genes (STING) lead to a severe autoinflammatory disease called STING-associated vasculopathy with onset in infancy that is associated with enhanced expression of interferon-stimulated gene transcripts.

Objective: The goal of this study was to analyze the phenotype of a new mouse model of STING hyperactivation and the role of type I interferons in this system.

Methods: We generated a knock-in model carrying an amino acid substitution (V154M) in mouse STING, corresponding to a recurrent mutation seen in human patients with STING-associated vasculopathy with onset in infancy. Read More

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http://dx.doi.org/10.1016/j.jaci.2018.04.034DOI Listing
February 2019
24 Reads

[New and traditional directions in the biology and management of childhood acute lymphoblastic leukemia].

Orv Hetil 2018 May;159(20):786-797

Genetikai, Sejt- és Immunbiológiai Intézet, Semmelweis Egyetem, Általános Orvostudományi Kar Budapest.

Owing to clinical trials and improvement over the past few decades, the majority of children with acute lymphoblastic leukemia (ALL) survive by first-line chemotherapy and combat with the problems of returning to community. However, many patients may have severe acute or late therapeutic side effects, and the survival rate in some groups (e.g. Read More

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http://dx.doi.org/10.1556/650.2018.31059DOI Listing
May 2018
47 Reads

Modern specific features and therapy of psoriasis and arthropathic psoriasis courses.

Wiad Lek 2018;71(2 pt 1):322-325

Danylo Halytsky Lviv National Medical University, Lviv, Ukraine.

Objective: Introduction:Psoriasis affects about 2% of population. In 30-40% of occurrences arthropathic psoriasis (AP) is diagnosed and it leads to 11-19% of disability cases development. Recent studies have shown that psoriasis is often synergistically combined with herpesvirus of the 1st and 2nd type, which, according to many scientists, determines the severity, frequency of recurrences, and the course of this dermatosis. Read More

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January 2018
3 Reads

B-cell differentiation and IL-21 response in SCID patients after hematopoietic stem cell transplantation.

Blood 2018 Jun 4;131(26):2967-2977. Epub 2018 May 4.

Division of Pediatric Hematology-Oncology, Boston Children's Hospital, Boston, MA.

Allogeneic hematopoietic stem cell transplant (HSCT) typically results in donor T-cell engraftment and function in patients with severe combined immunodeficiency (SCID), but humoral immunity, particularly when using donors other than matched siblings, is variable. B-cell function after HSCT for SCID depends on the genetic cause, the use of pre-HSCT conditioning, and whether donor B-cell chimerism is achieved. Patients with defects in or undergoing HSCT without conditioning often have poor B-cell function post-HSCT, perhaps as a result of impairment of IL-21 signaling in host-derived B cells. Read More

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http://dx.doi.org/10.1182/blood-2017-10-809822DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6024640PMC
June 2018
10 Reads

Immunogenicity and Immunological Memory Induced by the 13-Valent Pneumococcal Conjugate Followed by the 23-Valent Polysaccharide Vaccine in HIV-Infected Adults.

J Infect Dis 2018 Jun;218(1):26-34

Immunobiology and Vaccinology Research Laboratory, Aghia Sophia Children's Hospital.

Background: Vaccine-induced memory B-cell (MBC) subsets have distinct roles in the establishment of protective immunity; MBCs expressing nonswitched immunoglobulin M (IgM+ MBCs) replenish the MBC pool, whereas MBCs expressing isotype-switched immunoglobulin (sIg+ MBCs) differentiate into plasma cells upon antigen reencounter. We investigated immunogenicity and MBCs induced by combined 13-valent pneumococcal conjugate vaccine (PCV13) and 23-valent pneumococcal polysaccharide vaccine (PPV23) in human immunodeficiency virus (HIV)-infected adults.

Methods: Forty HIV-seropositive adults receiving ART with undetectable viral loads were enrolled. Read More

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http://dx.doi.org/10.1093/infdis/jiy135DOI Listing
June 2018
2 Reads

Multispectral fluorescence-activated cell sorting of B and T cell subpopulations from equine peripheral blood.

Vet Immunol Immunopathol 2018 May 23;199:22-31. Epub 2018 Mar 23.

Baker Institute for Animal Health, College of Veterinary Medicine, Cornell University, Ithaca, NY, 14850, USA.

Immune phenotyping of equine peripheral blood mononuclear cells (PBMC) is commonly described by single or double marker labeling, which limits complex phenotypic descriptions and subpopulation identification. Our objective was to develop a new multispectral flow cytometry protocol to identify and sort equine lymphocyte subpopulations using commercially available, pre-conjugated monoclonal antibodies to cell surface markers. Two clones of anti-equine CD8 (CVS8 and CVS21) were compared in combination with CD3. Read More

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http://dx.doi.org/10.1016/j.vetimm.2018.03.010DOI Listing