[HTML][HTML] T cell receptor (TCR) signaling in health and disease

K Shah, A Al-Haidari, J Sun, JU Kazi - Signal transduction and targeted …, 2021 - nature.com
Interaction of the T cell receptor (TCR) with an MHC-antigenic peptide complex results in
changes at the molecular and cellular levels in T cells. The outside environmental cues are …

Cytokine storm syndrome

RQ Cron, G Goyal, WW Chatham - Annual Review of Medicine, 2023 - annualreviews.org
Cytokine storm syndrome (CSS), which is frequently fatal, has garnered increased attention
with the ongoing coronavirus pandemic. A variety of hyperinflammatory conditions …

[HTML][HTML] The diagnostic approach to monogenic very early onset inflammatory bowel disease

HH Uhlig, T Schwerd, S Koletzko, N Shah… - Gastroenterology, 2014 - Elsevier
Patients with a diverse spectrum of rare genetic disorders can present with inflammatory
bowel disease (monogenic IBD). Patients with these disorders often develop symptoms …

[HTML][HTML] Hematopoietic stem cell transplantation in primary immunodeficiency diseases: current status and future perspectives

R Castagnoli, OM Delmonte, E Calzoni… - Frontiers in …, 2019 - frontiersin.org
Primary immunodeficiencies (PID) are disorders that for the most part result from mutations
in genes involved in immune host defense and immunoregulation. These conditions are …

Primary immunodeficiency and autoimmunity: a comprehensive review

L Amaya-Uribe, M Rojas, G Azizi, JM Anaya… - Journal of …, 2019 - Elsevier
The primary immunodeficiency diseases (PIDs) include many genetic disorders that affect
different components of the innate and adaptive responses. The number of distinct genetic …

Familial and acquired hemophagocytic lymphohistiocytosis

GE Janka - Annual review of medicine, 2012 - annualreviews.org
Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening syndrome in which an
uncontrolled and ineffective immune response, triggered in most cases by infectious agents …

Hemophagocytic lymphohistiocytosis: pathogenesis and treatment

GE Janka, K Lehmberg - Hematology 2013, the American …, 2013 - ashpublications.org
Hemophagocytic lymphohistiocytosis (HLH) is not an independent disease but rather a life-
threatening clinical syndrome that occurs in many underlying conditions and in all age …

Practice parameter for the diagnosis and management of primary immunodeficiency

FA Bonilla, DA Khan, ZK Ballas, J Chinen… - Journal of Allergy and …, 2015 - Elsevier
The American Academy of Allergy, Asthma & Immunology (AAAAI) and the American
College of Allergy, Asthma & Immunology (ACAAI) have jointly accepted responsibility for …

SLAM family receptors and SAP adaptors in immunity

JL Cannons, SG Tangye… - Annual review of …, 2011 - annualreviews.org
The signaling lymphocyte activation molecule (SLAM)-associated protein, SAP, was first
identified as the protein affected in most cases of X-linked lymphoproliferative (XLP) …

Hemophagocytic syndromes—an update

GE Janka, K Lehmberg - Blood reviews, 2014 - Elsevier
Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory
syndrome and not an independent disease. HLH represents the extreme end of a severe …