Hemophagocytic lymphohistiocytosis

TS Ponnatt, CM Lilley, KM Mirza - Archives of Pathology & …, 2022 - meridian.allenpress.com
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 …

Hemophagocytic syndromes and infection.

DN Fisman - Emerging infectious diseases, 2000 - ncbi.nlm.nih.gov
Hemophagocytic lymphohistiocytosis (HLH) is an unusual syndrome characterized by fever,
splenomegaly, jaundice, and the pathologic finding of hemophagocytosis (phagocytosis by …

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 …

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 …

[HTML][HTML] Munc13-4 is essential for cytolytic granules fusion and is mutated in a form of familial hemophagocytic lymphohistiocytosis (FHL3)

J Feldmann, I Callebaut, G Raposo, S Certain, D Bacq… - Cell, 2003 - cell.com
Secretion of cytolytic granules content at the immunological synapse is a highly regulated
process essential for lymphocyte cytotoxicity. This process requires the rapid transfer of …

Familial hemophagocytic lymphohistiocytosis type 5 (FHL-5) is caused by mutations in Munc18-2 and impaired binding to syntaxin 11

U Zur Stadt, J Rohr, W Seifert, F Koch, S Grieve… - The American Journal of …, 2009 - cell.com
Rapid intracellular transport and secretion of cytotoxic granules through the immunological
synapse requires a balanced interaction of several proteins. Disturbance of this highly …

Linkage of familial hemophagocytic lymphohistiocytosis (FHL) type-4 to chromosome 6q24 and identification of mutations in syntaxin 11

U zur Stadt, S Schmidt, B Kasper… - Human molecular …, 2005 - academic.oup.com
Familial hemophagocytic lymphohistiocytosis (FHL) is a rare autosomal recessive disorder
characterized by hyperactive phagocytes and defects in natural killer cell function. It has …

Hemophagocytic lymphohistiocytosis: advances in pathophysiology, diagnosis, and treatment

S Chandrakasan, AH Filipovich - The Journal of pediatrics, 2013 - jpeds.com
Shanmuganathan Chandrakasan, MD, and Alexandra H. Filipovich, MD Hemophagocytic
lymphohistiocytosis (HLH) is a potentially life-threatening disorder characterized by immune …

Clinical features and treatment strategies of Epstein–Barr virus-associated hemophagocytic lymphohistiocytosis

S Imashuku - Critical reviews in oncology/hematology, 2002 - Elsevier
Epstein–Barr virus (EBV) is the major triggering factor producing hemophagocytic syndrome
or hemophagocytic lymphohistiocytosis (HLH). In this review, diagnostic problems, clinical …

Pathophysiology and epidemiology of hemophagocytic lymphohistiocytosis

CE Allen, KL McClain - Hematology 2014, the American …, 2015 - ashpublications.org
Hemophagocytic lymphohistiocytosis (HLH) is a syndrome characterized by extreme
immune activation, resulting in pathologic inflammation. The diagnosis includes a spectrum …