Results 61 to 70 of about 13,338 (182)

An Atypical Skin Presentation of Adult‐Onset Still's Disease With Histological Description: A Case Report

Journal of Cutaneous Pathology, EarlyView.
ABSTRACT Adult‐onset Still's disease (AOSD) is an auto inflammatory disorder with a variable clinical presentation, and without a pathognomonic diagnostic test, characterized by high spiking fever, arthralgia/arthritis, a suggestive skin rash, elevated white blood cell count 10 G/L (> 80% neutrophils), elevated ferritin, with glycosylated ferritin < 20%
M. Nordmann, T. Mazza, S. Menzinger, G. Kaya, S. Bogiatzi, S. Vandenberghe‐Dürr, M. Blanchard, J. Stirnemann   +7 more
wiley   +1 more source

Inborn errors of immunity in children with neuroinflammation

Developmental Medicine &Child Neurology, EarlyView.
Abstract Inborn errors of immunity (IEIs), an expanding group of monogenic disorders with diverse clinical manifestations, are increasingly recognized to include neuroinflammatory disease. Examples of diseases included under this umbrella are Aicardi–Goutières syndrome, deficiency of adenosine deaminase 2, familial haemophagocytic lymphohistiocytosis ...
Eppie M Yiu, Dilan Dissanayake, Jessie Cunningham, Helen Branson, Ori Scott, E Ann Yeh   +5 more
wiley   +1 more source

Third Allogeneic Stem Cell Transplantation in Children: A Multicenter Analysis From the Spanish GETH‐TC Pediatric Group

European Journal of Haematology, EarlyView.
ABSTRACT Third allogeneic hematopoietic stem cell transplantation (HSCT3) is rarely performed in pediatric patients and is associated with high toxicity and mortality. Data on outcomes in this setting remain scarce. We conducted a retrospective multicenter study within the Spanish GETH‐TC Pediatric group including 29 children and adolescents who ...
Luisa Sisinni, Sara Vinagre Enríquez, Marta Villalon Herrera, Mercedes Plaza Fornieles, Iván López Torija, Andrea Urtasun Erburu, Laura Alonso García, Alexandra Regueiro García, Maria Trabazo del Castillo, Eduardo J. Bardón‐Cancho, Odelaisy León‐Triana, Carla Miñarro Chacón, José Luís Fuster Soler, Antonio Pérez‐Martínez, Pascual Balsalobre López, Marta Gonzalez‐Vicent, Cristina Díaz de Heredia, on behalf of the GETH‐TC Pediatric Working Group   +17 more
wiley   +1 more source

Prognostic Value of Skin Lesion Characteristics in Subcutaneous Panniculitis‐Like T‐Cell Lymphoma: A Retrospective Cohort Study Assessing the Applicability of T Category of the TNM Classification for Primary Cutaneous Lymphomas Other Than Mycosis Fungoides and Sézary Syndrome

International Journal of Dermatology, EarlyView.
Subcutaneous panniculitis‐like T‐cell lymphoma. Survival outcomes according to the presence of hemophagocytic lymphohistiocytosis (HLH) and T category. Relapse‐free survival was inferior in patients presenting with generalized (T3) skin lesions. Overall survival was poor in patients with HLH. Relapse‐free survival was also reduced in patients with HLH.
Myoung Eun Choi, Joon Min Jung, Soo Hyun Jeon, Chong Hyun Won, Sung Eun Chang, Mi Woo Lee, Woo Jin Lee   +6 more
wiley   +1 more source

Toxoplasmosis Beyond Transplantation: Diagnostic and Prevention Challenges in a Patient Receiving Targeted Immunomodulators

Transplant Infectious Disease, EarlyView.
ABSTRACT Toxoplasmosis has long been recognized as a serious complication in immunocompromised host, particularly those with advanced HIV/AIDS, hematopoietic stem‐cell transplantation (HSCT), solid‐organ transplant (SOT), and hematological malignancies. The rapid expansion of targeted immunomodulators, including chimeric antigen receptor T‐cell (CAR‐T)
Joy Mouanes‐Abelin, Christelle Pomares, José G. Montoya, Morgane Pondrom, Lucille DE MARIA, Andrea J. Zimmer, Carlos A. Gomez   +6 more
wiley   +1 more source

Atypical Clinical Course of Griscelli Syndrome Type 2 With Primarily Neurologic Presentation and Adult‐Onset in a 46‐Year‐Old Male

American Journal of Medical Genetics Part A, Volume 200, Issue 8, Page 1850-1855, August 2026.
ABSTRACT Griscelli Syndrome Type 2 (GS2) is a rare autosomal recessive disorder caused by pathogenic mutations in the RAB27A gene. Typically, it is characterized by cutaneous hypopigmentation, immunodeficiency, with or without neurological abnormalities secondary to hemophagocytic lymphohistiocytosis (HLH). Without treatment, GS2 often results in fatal
Dzhoy Papingi, Michael Kutsche, Helena Lichtenfeld, Fanny Kortüm, Angela Abicht, Laura Herrmann, Theresia Herget   +6 more
wiley   +1 more source

Secondary hemophagocytic lymphohistiocytosis in children with brucellosis: report of three cases

Journal of Infection in Developing Countries, 2015
Brucellosis is a systemic zoonotic infectious disease that may cause fever, fatigue, sweating, arthritis, hepatosplenomegaly, cytopenia, and lymphadenopathy. It continues to be an important health problem worldwide.
Yöntem Yaman, Salih Gözmen, Ahmet Kağan Özkaya, Yeşim Oymak, Hurşit Apa, Canan Vergin, İlker Devrim   +6 more
doaj   +1 more source

Immune Dysregulation in Branched Chain Organic Acidemias

Journal of Inherited Metabolic Disease, Volume 49, Issue 4, July 2026.
ABSTRACT Organic acidemias (OAs) are a group of inherited disorders, most commonly caused by defects in mitochondrial enzymes involved in amino acid and fatty acid metabolism. While they characteristically present with metabolic and neurological crises, growing evidence reveals a significant burden of chronic immune dysregulation in some disorders and ...
Abdul L. Shakerdi, Jack Drda, Justin A. Dutta, Jerry Vockley   +3 more
wiley   +1 more source

Fulminant hemophagocytic lymphohistiocytosis induced by pandemic A (H1N1) influenza: a case report

Journal of Medical Case Reports, 2011
Introduction Hemophagocytic lymphohistiocytosis induced by viral diseases is a well recognized entity. Severe forms of H5N1 influenza are known to be associated with symptoms very similar to a reactive hemophagocytic syndrome.
Wacrenier Agnès, Guerry Mary-Jane, Cornelius Aurélie, Willekens Christophe, Fourrier François   +4 more
doaj   +1 more source

Incidence and Survival of Hemophagocytic Lymphohistiocytosis Over Two Decades: A Population‐Based Study

European Journal of Haematology, Volume 117, Issue 1, Page 210-218, July 2026.
ABSTRACT Adult hemophagocytic lymphohistiocytosis (HLH) is a rare, life‐threatening syndrome triggered by various conditions. A nationwide study of the incidence and outcomes of HLH in Denmark over 23 years (2000–2023) was performed. Adults (≥ 18 years) with HLH and triggering diseases were identified in the Danish National Patient Registry and/or the ...
Mads Okkels Birk Lorenzen, Martin Jensen, Rasmus Rask Kragh Jørgensen, Paw Jensen, Henrik Frederiksen, Dennis L. Hansen, Daniel El Fassi, Morten Nørgaard Andersen, Tarec Christoffer El‐Galaly   +8 more
wiley   +1 more source