Results 51 to 60 of about 16,441 (215)

A 9month-old-boy with atypical hemophagocytic lymphohistiocytosis

Mediterranean Journal of Hematology and Infectious Diseases, 2017
Hemophagocytic lymphohistiocytosis is a life-threatening hyperinflammation caused by uncontrolled proliferation of activated lymphocytes and histiocytes. Often, Hemophagocytic lymphohistiocytosis is an acquired syndrome. We report a case of a 9 month-old-
Monia Ouederni, MONIA BEN KHALED, Samia Rekaya, Ilhem Ben Fraj, Fethi Mellouli, Mohamed Bejaoui   +5 more
doaj   +1 more source

Cerebral Involvement of Hemophagocytic Lymphohistiocytosis in Griscelli Syndrome

, 2019
Type II Griscelli Syndrome (GS) is caused by a mutation in the RAB27A gene and usually manifests with silvery-gray hair, immune deficiency and the development of hemophagocytic lymphohistiocytosis (HLH).
Ersin Töret, Aksoylar, Serap, Oymak, Yeşim, Tuba Hilkay Karapınar, Yılmaz Ay, Ay, Yılmaz, Yeşim Oymak, Toret, Ersin, Serap Aksoylar, Karapınar, Tuba Hilkay   +9 more
core   +1 more source

A Multifaceted Interplay Among Hemophagocytosis, Interleukin‐18, and Type I Interferon Distinguishes Still Disease From Other Autoinflammatory Diseases

Arthritis &Rheumatology, EarlyView.
Objective The unknown pathophysiology and the lack of specific features for systemic juvenile idiopathic arthritis and adult‐onset Still disease (collectively known as Still disease; SD) delay diagnosis and appropriate treatment. The goal of this study was to identify features and mechanisms that distinguish SD from other systemic autoinflammatory ...
Yvonne M. Mueller, Charlotte Girard, Harmen J. G. van de Werken, Stefan J. Erkeland, Dwin G. B. Grashof, Laurie Vaillant, Renske J. H. den Dekker, Iris van Zuijen, Sharon Veenbergen, Fanny Coffin, Eric Bindels, Yvan Jamilloux, Katerina Laskari, Vassili Soumelis, Bruno Fautrel, Cem Gabay, Peter D. Katsikis   +16 more
wiley   +1 more source

CLINICAL CASE OF TOCILIZUMAB THERAPY IN A PATIENT WITH SYSTEMIC JUVENILE IDIOPATHIC ARTHRITIS

Педиатрическая фармакология, 2013
The article presents a case of successful application of a monoclonal antibodies drug to interleukin 6 receptors (tocilizumab) at severe systemic juvenile idiopathic arthritis with the development of secondary hemophagocytic syndrome.
E. I. Alexeeva, E. I. Alexeeva, R. V. Denisova, T. V. Sleptsova   +3 more
doaj   +1 more source

From Interferon Signature to the Clinical Landscape: Type I Interferonopathies

Arthritis &Rheumatology, Accepted Article.
Background Type I interferonopathies are heterogeneous diseases driven by dysregulated IFN‐I signaling. Diagnosis is challenging due to clinical/molecular variability and the need for IFN‐I quantification. Objective To characterize the clinical, immunological, genetic, molecular profiles of patients with suspected enhanced IFN‐I signaling, and assess ...
Ismail Yaz, Seza Ozen, Hacer N. Bildik, Canberk Ipsir, Dilara Unal, Saliha Esenboga, Begum Cicek, Mehmet E. Seker, Fatima Aerts‐Kaya, Seher Sener, Mehmet O. Erkan, Hanife Avci, Deniz Cagdas, Ilhan Tezcan   +13 more
wiley   +1 more source

Hemophagocytic syndrome.

Saudi medical journal, 2016
Full text is available as a scanned copy of the original print version.
T M, Owaidah, S H, Khalil, M M, Mustafa, M, Ayas   +3 more
openaire   +3 more sources

Efficacy, safety and cost‐effectiveness of CAR‐T therapy

British Journal of Clinical Pharmacology, EarlyView.
CAR T‐cells demonstrate high efficacy in blood cancers, including ALL, MM and DLBCL. Innovations target solid tumours despite challenges such as antigen escape. Combination therapies enhance the delivery and infiltration of CAR T cells. Toxicity, cost and resistance remain major barriers to clinical use.
Emina Karahmet Sher, Amina Džidić‐Krivić, Emma Pinjic, Nejra Selak, Kanita Omerbasic, Alexandar Chupin, Andrej Belancic, Almir Fajkic   +7 more
wiley   +1 more source

Ruxolitinib Pharmacokinetics and Exposure–Toxicity Relationship in Hematologic Malignancies and Immune‐Mediated Diseases: A Prospective Observational Study

Clinical Pharmacology &Therapeutics, EarlyView.
Ruxolitinib pharmacokinetics (PK) has been characterized in clinical trials but remains poorly documented in real‐world practice. This project aimed to investigate ruxolitinib PK in routine clinical practice, identify factors driving its variability, and explore exposure–response relationships to assess the potential role of therapeutic drug monitoring.
Jérémie Tachet, Sara C. Meyer, Francesco Grandoni, Emmanuel Haefliger, Monika Nagy‐Hulliger, Jörg P. Halter, Jakob R. Passweg, David Haefliger, Carine Bardinet, Laurent A. Decosterd, Monia Guidi, François R. Girardin   +11 more
wiley   +1 more source

Scrub Typhus Associated Secondary Hemophagocytic Lymphohistiocytosis in Pediatric Intensive Care Unit

Journal of Nobel Medical College, 2023
Background: Scrub Typhus is febrile illness caused by orientia tsutsugamusi and presents with multiorgan involvement. It can cause multiorgan failure resulting in acute respiratory distress syndrome, acute kidney Injury and hemophagocytic ...
Sandip Kumar Singh, Shishir Jha, Rajnish Mishra, Sajan Kumar Thakur, Suja Gurung, Aliza Bajracharya   +5 more
doaj   +1 more source

Family hemophagocytic lymphohistiocytosis: (2 clinical cases in one family)

Журнал инфектологии, 2019
Family hemophagocytic lymphohistiocytosis (hemophagocytic syndrome) is a rare hereditary disease, which is based on a disturbance of the regulation of the immune response, leading to proliferation and activation of histiocytes, phagocytosis of peripheral
N. A. Efremova, L. G. Goryacheva, S. P. Kaplina, V. A. Greshnyakova, A. A. Osipova, T. A. Bykova   +5 more
doaj   +1 more source