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 …

Controlling natural killer cell responses: integration of signals for activation and inhibition

EO Long, H Sik Kim, D Liu, ME Peterson… - Annual review of …, 2013 - annualreviews.org
Understanding how signals are integrated to control natural killer (NK) cell responsiveness
in the absence of antigen-specific receptors has been a challenge, but recent work has …

Natural killer cell deficiency

JS Orange - Journal of Allergy and Clinical Immunology, 2013 - Elsevier
Natural killer (NK) cells are part of the innate immune defense against infection and cancer
and are especially useful in combating certain viral pathogens. The utility of NK cells in …

Chaperoning SNARE folding and assembly

Y Zhang, FM Hughson - Annual review of biochemistry, 2021 - annualreviews.org
SNARE proteins and Sec1/Munc18 (SM) proteins constitute the core molecular engine that
drives nearly all intracellular membrane fusion and exocytosis. While SNAREs are known to …

Chemoimmunotherapy for hemophagocytic lymphohistiocytosis: long-term results of the HLH-94 treatment protocol

H Trottestam, AC Horne, M Arico… - Blood, The Journal …, 2011 - ashpublications.org
Hemophagocytic lymphohistiocytosis (HLH) used to have a dismal prognosis. We report the
final results of HLH-94, the largest prospective diagnostic/therapeutic HLH study so far. The …

Innate immunosenescence: effect of aging on cells and receptors of the innate immune system in humans

R Solana, R Tarazona, I Gayoso, O Lesur… - Seminars in …, 2012 - Elsevier
Components of the innate immune response, including neutrophils and macrophages, are
the first line of defense against infections. Their role is to initiate an inflammatory response …

[HTML][HTML] The life cycle of platelet granules

A Sharda, R Flaumenhaft - F1000Research, 2018 - ncbi.nlm.nih.gov
Platelet granules are unique among secretory vesicles in both their content and their life
cycle. Platelets contain three major granule types—dense granules, α-granules, and …

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 …

The road to lysosome‐related organelles: Insights from Hermansky‐Pudlak syndrome and other rare diseases

SL Bowman, J Bi‐Karchin, L Le, MS Marks - Traffic, 2019 - Wiley Online Library
Lysosome‐related organelles (LROs) comprise a diverse group of cell type‐specific,
membrane‐bound subcellular organelles that derive at least in part from the endolysosomal …

Horror Autoinflammaticus: The Molecular Pathophysiology of Autoinflammatory Disease

SL Masters, A Simon, I Aksentijevich… - Annual review of …, 2009 - annualreviews.org
The autoinflammatory diseases are characterized by seemingly unprovoked episodes of
inflammation, without high-titer autoantibodies or antigen-specific T cells. The concept was …