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 …

Pediatric hemophagocytic lymphohistiocytosis

SW Canna, RA Marsh - Blood, The Journal of the American …, 2020 - ashpublications.org
Hemophagocytic lymphohistiocytosis (HLH) is a syndrome describing patients with severe
systemic hyperinflammation. Characteristic features include unremitting fever, cytopenias …

Revised diagnostic criteria and classification for the autoimmune lymphoproliferative syndrome (ALPS): report from the 2009 NIH International Workshop

JB Oliveira, JJ Bleesing, U Dianzani… - Blood, The Journal …, 2010 - ashpublications.org
Lymphadenopathy in children for which no infectious or malignant cause can be ascertained
constitutes a challenging diagnostic dilemma. Autoimmune lymphoproliferative syndrome …

Clinical similarities and differences of patients with X-linked lymphoproliferative syndrome type 1 (XLP-1/SAP deficiency) versus type 2 (XLP-2/XIAP deficiency)

JP Schmid, D Canioni, D Moshous… - Blood, The Journal …, 2011 - ashpublications.org
X-linked lymphoproliferative syndromes (XLP) are primary immunodeficiencies
characterized by a particular vulnerability toward Epstein-Barr virus infection, frequently …

Human immunity against EBV—lessons from the clinic

SG Tangye, U Palendira, ESJ Edwards - Journal of Experimental …, 2017 - rupress.org
The mammalian immune system has evolved over many millennia to be best equipped to
protect the host from pathogen infection. In many cases, host and pathogen have coevolved …

Signaling pathways involved in the T‐cell‐mediated immunity against Epstein‐Barr virus: Lessons from genetic diseases

S Latour, A Fischer - Immunological Reviews, 2019 - Wiley Online Library
Primary immunodeficiencies (PIDs) provide researchers with unique models to understand
in vivo immune responses in general and immunity to infections in particular. In humans …

[HTML][HTML] Histiocytic disorders: recent insights into pathophysiology and practical guidelines

A Filipovich, K McClain, A Grom - Biology of Blood and Marrow …, 2010 - Elsevier
The Histiocytoses are defined as non-malignant disorders due to abnormal accumulation
and behavior of cells of the mononuclear phagocytic system. The best known histiocytoses …

Epstein–Barr virus and hemophagocytic lymphohistiocytosis

RA Marsh - Frontiers in immunology, 2018 - frontiersin.org
Epstein–Barr virus (EBV) is a ubiquitous virus that infects nearly all people worldwide
without serious sequela. However, for patients who have genetic diseases which predispose …

XIAP deficiency: a unique primary immunodeficiency best classified as X-linked familial hemophagocytic lymphohistiocytosis and not as X-linked lymphoproliferative …

RA Marsh, L Madden, BJ Kitchen… - Blood, The Journal …, 2010 - ashpublications.org
X-linked inhibitor of apoptosis (XIAP) deficiency, caused by BIRC4 mutations, is described to
cause X-linked lymphoproliferative disease (XLP) phenotypes. However, compared with …

Primary immunodeficiencies associated with EBV disease

JI Cohen - Epstein Barr Virus Volume 1: One Herpes Virus: Many …, 2015 - Springer
Epstein-Barr virus (EBV) infects nearly all humans and usually is asymptomatic, or in the
case of adolescents and young adults, it can result in infectious mononucleosis. EBV …