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 …

Hemophagocytic syndromes—an update

GE Janka, K Lehmberg - Blood reviews, 2014 - Elsevier
Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory
syndrome and not an independent disease. HLH represents the extreme end of a severe …

Salvage therapy of refractory hemophagocytic lymphohistiocytosis with alemtuzumab

RA Marsh, CE Allen, KL McClain… - Pediatric blood & …, 2013 - Wiley Online Library
Background Hemophagocytic lymphohistiocytosis (HLH) is a life‐threatening
hyperinflammatory syndrome that remains difficult to treat. Even with current standard HLH …

How I treat Langerhans cell histiocytosis

CE Allen, S Ladisch, KL McClain - Blood, The Journal of the …, 2015 - ashpublications.org
Abstract “Langerhans cell histiocytosis”(LCH) describes a spectrum of clinical presentations
ranging from a single bone lesion or trivial skin rash to an explosive disseminated disease …

Reduced-intensity conditioning significantly improves survival of patients with hemophagocytic lymphohistiocytosis undergoing allogeneic hematopoietic cell …

RA Marsh, G Vaughn, MO Kim, D Li… - Blood, The Journal …, 2010 - ashpublications.org
Recent experience suggests that reduced-intensity conditioning (RIC) regimens can
improve the outcomes of patients with hemophagocytic lymphohistiocytosis (HLH) …

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 …

Hemophagocytic lymphohistiocytosis: clinical presentations and diagnosis

KA Risma, RA Marsh - The Journal of Allergy and Clinical Immunology: In …, 2019 - Elsevier
Hemophagocytic lymphohistiocytosis (HLH) is an overwhelming clinical syndrome
associated with extreme immune activation. Familial HLH is caused by autosomal-recessive …

Familial haemophagocytic lymphohistiocytosis: advances in the genetic basis, diagnosis and management

C Gholam, S Grigoriadou, KC Gilmour… - Clinical & …, 2011 - academic.oup.com
Familial haemophagocytic lymphohistiocytosis (FHL) is a rare autosomal recessive disorder
of immune dysregulation associated with uncontrolled T cell and macrophage activation and …

Reduced-intensity conditioning for hematopoietic cell transplant for HLH and primary immune deficiencies

CE Allen, R Marsh, P Dawson… - Blood, The Journal …, 2018 - ashpublications.org
Allogeneic hematopoietic cell transplantation (HCT) with myeloablative conditioning for
disorders associated with excessive inflammation such as hemophagocytic …

Review of haemophagocytic lymphohistiocytosis

HR Freeman, AV Ramanan - Archives of disease in childhood, 2011 - adc.bmj.com
Haemophagocytic lymphohistiocytosis (HLH) describes a clinical syndrome of
hyperinflammation resulting in an uncontrolled and ineffective immune response. It may …