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A Systematic Review on Predisposition to Lymphoid (B and T cell) Neoplasias in Patients With Primary Immunodeficiencies and Immune Dysregulatory Disorders (Inborn Errors of Immunity)
Conclusions Though this is not a comprehensive summary of malignancies in PIDDs, or even lymphoproliferative disease in this area, this review summarizes the Medline-indexed published reports of B and T lymphomas in patients with PIDDs. This report highlights the diversity of malignant lymphoproliferative disorders in setting of PIDDs, and its associated challenges of diagnosis and treatment. The pathological classification and nomenclature for the lymphoid malignancies with variably reported and postulated underlying mechanisms were inconsistent and inadequate for many of these published reports. A wide range of treatmen...
Source: Frontiers in Immunology - April 15, 2019 Category: Allergy & Immunology Source Type: research

Case Study: Mechanism for Increased Follicular Helper T Cell Development in Activated PI3K Delta Syndrome
This study was carried out after written informed consent from all subjects. All subjects gave written informed consent in accordance with the Declaration of Helsinki. The protocol was approved by the Stanford University and UCLA Institutional Review Boards. Author Contributions MB provided patient care and obtained IRB approval. RO prepared histology images. MB, TT, and RB designed the research. TT and LP conducted experiments and analyzed data. MB made the molecular model. MB and TT wrote the manuscript. Funding Funding for this work came from the Jeffrey Modell Foundation and from the NIH/NIGMS (R01 GM110482 to MB)....
Source: Frontiers in Immunology - April 11, 2019 Category: Allergy & Immunology Source Type: research

The Pivotal Role of Regulatory T Cells in the Regulation of Innate Immune Cells
This study was provided by the Canadian Institutes of Health Research and the Manitoba Health Research Council. Conflict of Interest Statement The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. References 1. Sprent J, Kishimoto H. The thymus and central tolerance. Philos Trans R Soc Lond B Biol Sci. (2001) 356:609–16. doi: 10.1098/rstb.2001.0846 PubMed Abstract | CrossRef Full Text | Google Scholar 2. Sakaguchi S, Wing K, Miyara M. Regulatory T cells - a brief history and perspective...
Source: Frontiers in Immunology - April 8, 2019 Category: Allergy & Immunology Source Type: research

Clinical Spectrum of Patients with Pathogenic Variant of STAT3 conferring Gain-of-Function: A Mimic of Autoimmune Lymphoproliferative Syndrome
Autoimmune lymphoproliferative syndrome (ALPS) is a rare genetic disorder caused by defective fas-mediated apoptosis. Patients often present in childhood with lymphoproliferation, splenomegaly and multilineage cytopenias (Price et al. Natural history of autoimmune lymphoproliferative syndrome associated with FAS gene mutations. Blood 2014). Though mutations in the FAS gene account for the majority of cases, an estimated 20% of patients who have no defined genetic cause are classified as ALPS-U (Shah et al. Autoimmune lymphoproliferative syndrome: an update and review of the literature. Current allergy and asthma reports 20...
Source: Blood - November 21, 2018 Category: Hematology Authors: Constantine, G., Su, H., Folio, L., Milner, J., Rao, V. K. Tags: 203. Lymphocytes, Lymphocyte Activation, and Immunodeficiency, including HIV and Other Infections: Poster III Source Type: research

Autologous haematopoietic stem cell transplantation for aggressive multiple sclerosis: the Swedish experience
Conclusions HSCT is a very effective treatment of inflammatory active MS and can be performed with a high degree of safety at experienced centres.
Source: Journal of Neurology, Neurosurgery and Psychiatry - September 9, 2014 Category: Neurosurgery Authors: Burman, J., Iacobaeus, E., Svenningsson, A., Lycke, J., Gunnarsson, M., Nilsson, P., Vrethem, M., Fredrikson, S., Martin, C., Sandstedt, A., Uggla, B., Lenhoff, S., Johansson, J.-E., Isaksson, C., Hagglund, H., Carlson, K., Fagius, J. Tags: Immunology (including allergy), Multiple sclerosis Source Type: research