TY - JOUR
T1 - Adult haemophagocytic syndrome
AU - Ramos-Casals, Manuel
AU - Brito-Zerón, Pilar
AU - López-Guillermo, Armando
AU - Khamashta, Munther
AU - Bosch, Xavier
PY - 2014
Y1 - 2014
N2 - Haemophagocytic syndromes (haemophagocytic lymphohistiocytosis) have a wide range of causes, symptoms, and outcomes, but all lead to a hyperinflammatory response and organ damage-mainly reported in paediatric patients, but reports of adult presentation are increasing. Analysis of the genetic and molecular pathophysiology of these syndromes have improved the understanding of the crosstalk between lymphocytes and histiocytes and their regulatoty mechanisms. Clinical presentations with a broad differential diagnosis, and often life-threatening outcome, complicate the management, which might include supportive intensive care, immunosuppressive and biological treatments, or haemopoietic stem cell transplantation. Insufficient knowledge of these syndromes could contribute to poor prognosis. Early diagnosis is essential to initiate appropriate treatment and improve the quality of life and survival of patients with this challenging disorder.
AB - Haemophagocytic syndromes (haemophagocytic lymphohistiocytosis) have a wide range of causes, symptoms, and outcomes, but all lead to a hyperinflammatory response and organ damage-mainly reported in paediatric patients, but reports of adult presentation are increasing. Analysis of the genetic and molecular pathophysiology of these syndromes have improved the understanding of the crosstalk between lymphocytes and histiocytes and their regulatoty mechanisms. Clinical presentations with a broad differential diagnosis, and often life-threatening outcome, complicate the management, which might include supportive intensive care, immunosuppressive and biological treatments, or haemopoietic stem cell transplantation. Insufficient knowledge of these syndromes could contribute to poor prognosis. Early diagnosis is essential to initiate appropriate treatment and improve the quality of life and survival of patients with this challenging disorder.
UR - http://www.scopus.com/inward/record.url?scp=84899658785&partnerID=8YFLogxK
U2 - 10.1016/S0140-6736(13)61048-X
DO - 10.1016/S0140-6736(13)61048-X
M3 - Article
C2 - 24290661
AN - SCOPUS:84899658785
SN - 0140-6736
VL - 383
SP - 1503
EP - 1516
JO - Lancet
JF - Lancet
IS - 9927
ER -