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 …
forms. The familial form is associated with mutations in the perforin gene and both forms are …
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 …
uncontrolled and ineffective immune response, triggered in most cases by infectious agents …
Hemophagocytic lymphohistiocytosis: diagnosis, pathophysiology, treatment, and future perspectives
JW Verbsky, WJ Grossman - Annals of medicine, 2006 - Taylor & Francis
Hemophagocytic lymphohistiocytosis (HLH) is a rare life‐threatening disease in which the
immune system becomes overactive due to its inability to effectively respond to infections …
immune system becomes overactive due to its inability to effectively respond to infections …
Perforin gene defects in familial hemophagocytic lymphohistiocytosis
SE Stepp, R Dufourcq-Lagelouse, FL Deist, S Bhawan… - Science, 1999 - science.org
Familial hemophagocytic lymphohistiocytosis (FHL) is a rare, rapidly fatal, autosomal
recessive immune disorder characterized by uncontrolled activation of T cells and …
recessive immune disorder characterized by uncontrolled activation of T cells and …
Perforin expression in cytotoxic lymphocytes from patients with hemophagocytic lymphohistiocytosis and their family members
K Kogawa, SM Lee, J Villanueva… - Blood, The Journal …, 2002 - ashpublications.org
Mutations in the perforin gene have been described in some patients with hemophagocytic
lymphohistiocytosis (HLH), but the role of perforin defects in the pathogenesis of HLH …
lymphohistiocytosis (HLH), but the role of perforin defects in the pathogenesis of HLH …
Hemophagocytic lymphohistiocytosis: when the immune system runs amok
G Janka - Klinische Pädiatrie, 2009 - thieme-connect.com
Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening syndrome in which an
exaggerated but ineffective immune response leads to severe hyperinflammation. Key …
exaggerated but ineffective immune response leads to severe hyperinflammation. Key …
[HTML][HTML] Spectrum of perforin gene mutations in familial hemophagocytic lymphohistiocytosis
KG Ericson, B Fadeel, S Nilsson-Ardnor… - The American Journal of …, 2001 - cell.com
Familial hemophagocytic lymphohistiocytosis (FHL) is an autosomal recessive disease of
early childhood characterized by nonmalignant accumulation and multivisceral infiltration of …
early childhood characterized by nonmalignant accumulation and multivisceral infiltration of …
Genetic subtypes of familial hemophagocytic lymphohistiocytosis: correlations with clinical features and cytotoxic T lymphocyte/natural killer cell functions
E Ishii, I Ueda, R Shirakawa, K Yamamoto, H Horiuchi… - Blood, 2005 - ashpublications.org
Mutations of the perforin (PRF1) and MUNC13-4 genes distinguish 2 forms of familial
hemophagocytic lymphohistiocytosis (FHL2 and FHL3, respectively), but the clinical and …
hemophagocytic lymphohistiocytosis (FHL2 and FHL3, respectively), but the clinical and …
Hemophagocytic lymphohistiocytosis
Context.—Hemophagocytic lymphohistiocytosis (HLH) is a rare, life-threatening disorder of
immune regulation that can eventually result in end-organ damage and death. HLH is …
immune regulation that can eventually result in end-organ damage and death. HLH is …
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 …
threatening clinical syndrome that occurs in many underlying conditions and in all age …