The 'cytokine storm': molecular mechanisms and therapeutic prospects

R Karki, TD Kanneganti - Trends in immunology, 2021 - cell.com
Cytokine storm syndrome (CSS) has generally been described as a collection of clinical
manifestations resulting from an overactivated immune system. Cytokine storms (CSs) are …

Hemophagocytic lymphohistiocytosis

H Al-Samkari, N Berliner - Annual review of pathology …, 2018 - annualreviews.org
Hemophagocytic lymphohistiocytosis is a life-threatening disorder characterized by
unbridled activation of cytotoxic T lymphocytes, natural killer (NK) cells, and macrophages …

Hemophagocytic lymphohistiocytosis: review of etiologies and management

MR George - Journal of blood medicine, 2014 - Taylor & Francis
Hemophagocytic lymphohistiocytosis (HLH) covers a wide array of related life-threatening
conditions featuring ineffective immunity characterized by an uncontrolled …

HLH‐2004: diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis

JI Henter, AC Horne, M Aricó, RM Egeler… - Pediatric blood & …, 2007 - Wiley Online Library
In HLH‐94, the first prospective international treatment study for hemophagocytic
lymphohistiocytosis (HLH), diagnosis was based on five criteria (fever, splenomegaly …

Functional significance of the perforin/granzyme cell death pathway

JA Trapani, MJ Smyth - Nature Reviews Immunology, 2002 - nature.com
Perforin/granzyme-induced apoptosis is the main pathway used by cytotoxic lymphocytes to
eliminate virus-infected or transformed cells. Studies in gene-disrupted mice indicate that …

Hemophagocytic lymphohistiocytosis: an update on diagnosis and pathogenesis

FGN Rosado, AS Kim - American journal of clinical pathology, 2013 - academic.oup.com
Hemophagocytic lymphohistiocytosis (HLH) is a frequently fatal and likely underdiagnosed
disease involving a final common pathway of hypercytokinemia, which can result in end …

Treatment of hemophagocytic lymphohistiocytosis with HLH-94 immunochemotherapy and bone marrow transplantation

JI Henter, AC Samuelsson-Horne… - Blood, The Journal …, 2002 - ashpublications.org
Hemophagocytic lymphohistiocytosis (HLH) comprises familial (primary) hemophagocytic
lymphohistiocytosis (FHL) and secondary HLH (SHLH), both clinically characterized by …

Lymphocyte-mediated cytotoxicity

JH Russell, TJ Ley - Annual review of immunology, 2002 - annualreviews.org
Virtually all of the measurable cell-mediated cytotoxicity delivered by cytotoxic T
lymphocytes and natural killer cells comes from either the granule exocytosis pathway or the …

An animal model of hemophagocytic lymphohistiocytosis (HLH): CD8+ T cells and interferon gamma are essential for the disorder

MB Jordan, D Hildeman, J Kappler, P Marrack - Blood, 2004 - ashpublications.org
Hemophagocytic lymphohistiocytosis (HLH) is a rare disorder with familial and acquired
forms. The familial form is associated with mutations in the perforin gene and both forms are …

Molecular mechanisms of disease-causing missense mutations

S Stefl, H Nishi, M Petukh, AR Panchenko… - Journal of molecular …, 2013 - Elsevier
Genetic variations resulting in a change of amino acid sequence can have a dramatic effect
on stability, hydrogen bond network, conformational dynamics, activity and many other …