7 results match your criteria: "and the Berlin Institute of Health (BIH)[Affiliation]"

HSCT for systemic autoimmune diseases with neurologic involvement.

Handb Clin Neurol

August 2024

Department of Haematology, Oncology and Tumor Immunology, Charité-Universitätsmedizin Berlin, corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and the Berlin Institute of Health (BIH), Berlin, Germany.

Article Synopsis
  • * It is particularly beneficial for autoimmune conditions like multiple sclerosis and systemic lupus erythematosus, especially when neurological symptoms are present.
  • * The article reviews current research and outcomes, showing that many patients experience improvement or resolution of their neurological symptoms after receiving HSCT.
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The Role of Chimeric Antigen Receptor T-Cell Therapy in Immune-Mediated Neurological Diseases.

Ann Neurol

September 2024

Unit of Hematology and Bone Marrow Transplantation, IRCCS San Raffaele Scientific Hospital, Vita-Salute San Raffaele University, Milan, Italy.

Despite the use of 'high efficacy' disease-modifying therapies, disease activity and clinical progression of different immune-mediated neurological diseases continue for some patients, resulting in accumulating disability, deteriorating social and mental health, and high economic cost to patients and society. Although autologous hematopoietic stem cell transplant is an effective treatment modality, it is an intensive chemotherapy-based therapy with a range of short- and long-term side-effects. Chimeric antigen receptor T-cell therapy (CAR-T) has revolutionized the treatment of B-cell and other hematological malignancies, conferring long-term remission for otherwise refractory diseases.

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JAK inhibitor treatment for inborn errors of JAK/STAT signaling: An ESID/EBMT-IEWP retrospective study.

J Allergy Clin Immunol

January 2024

Institute for Immunodeficiency, Center for Chronic Immunodeficiency, Medical Center, Faculty of Medicine, University of Freiburg, Freiburg, Germany; Division of Pediatric Hematology and Oncology, Department of Pediatrics and Adolescent Medicine, Medical Center - University of Freiburg, Faculty of Medicine, University of Freiburg, Freiburg, Germany. Electronic address:

Article Synopsis
  • Inborn errors of immunity (IEI) with dysregulated JAK/STAT signaling can lead to immune dysfunction and infections, and while hematopoietic stem cell transplantation (HSCT) is a potential cure, initial outcomes were not promising.
  • This study evaluated the effectiveness of off-label JAK inhibitors (JAKi) as a treatment option for patients with hyperactive JAK/STAT signaling disorders at various European medical centers.
  • Results showed that 87% of patients with STAT1 gain of function and 90% with STAT3 gain of function saw symptom improvement, with mild adverse events reported; a significant portion of patients continued JAKi treatment successfully, and some proceeded to HSCT, achieving a 91%
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Granulomatosis with Polyangiitis (GPA) is a small vessel vasculitis typically associated with release of neutrophil extracellular traps (NETs) by activated neutrophils. In this study, we further aimed to investigate the contributions of neutrophils and NETs to the complex disease pathogenesis. We characterized the phenotype of neutrophils and their capacity to induce NETs.

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Blood biomarkers recommended for diagnosing and monitoring IgG4-related disease. Considerations from the ERN ReCONNET and collaborating partners.

Clin Exp Rheumatol

May 2022

Department of Rheumatology and Clinical Immunology, Charité-Universitätsmedizin Berlin, corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and the Berlin Institute of Health (BIH), Berlin, and Deutsches Rheuma-Forschungszentrum (DRFZ Berlin) - a Leibniz Institute, Autoimmunology Group, Berlin, Germany.

Immunoglobulin G4 (IgG4)-related disease (IgG4-RD) is a chronic, clinically heterogenous fibroinflammatory condition, characterised by an accumulation of IgG4 secreting plasma cells in affected tissues and associated with increased serum IgG4 concentrations. Despite a growing recognition of the disease among clinicians from different specialties worldwide, its indolent nature, lack of a single diagnostic test and ability to mimic other malignant, infective and inflammatory conditions, makes the diagnosis challenging. As treatment options evolve, biomarkers correlating with disease activity, predicting prognosis and response to treatment are deemed required.

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Over the past decades, hematopoietic stem cell transplantation (HSCT) has been evolving as specific treatment for patients with severe and refractory autoimmune diseases (ADs), where mechanistic studies have provided evidence for a profound immune renewal facilitating the observed beneficial responses. The intestinal microbiome plays an important role in host physiology including shaping the immune repertoire. The relationships between intestinal microbiota composition and outcomes after HSCT for hematologic diseases have been identified, particularly for predicting the mortality from infectious and non-infectious causes.

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Given its uniformly high expression on plasma cells, CD38 has been considered as a therapeutic target in patients with systemic lupus erythematosus (SLE). Herein, we investigate the distribution of CD38 expression by peripheral blood leukocyte lineages to evaluate the potential therapeutic effect of CD38-targeting antibodies on these immune cell subsets and to delineate the use of CD38 as a biomarker in SLE. We analyzed the expression of CD38 on peripheral blood leukocyte subsets by flow and mass cytometry in two different cohorts, comprising a total of 56 SLE patients.

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