Diagnostic evaluation of patients with suspected haemophagocytic lymphohistiocytosis.
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Diagnostic evaluation of patients with suspected haemophagocytic lymphohistiocytosis. / Lehmberg, Kai; Ehl, Stephan.
in: BRIT J HAEMATOL, Jahrgang 160, Nr. 3, 3, 2013, S. 275-287.Publikationen: SCORING: Beitrag in Fachzeitschrift/Zeitung › SCORING: Zeitschriftenaufsatz › Forschung › Begutachtung
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TY - JOUR
T1 - Diagnostic evaluation of patients with suspected haemophagocytic lymphohistiocytosis.
AU - Lehmberg, Kai
AU - Ehl, Stephan
PY - 2013
Y1 - 2013
N2 - Haemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory syndrome characterized by severely disturbed immune homeostasis. It can affect all age groups. Diagnostic evaluation of the patient with suspected HLH has to address three main questions: (i) does the patient have HLH? There is no simple diagnostic test, but a number of clinical and laboratory criteria define this clinical syndrome. (ii) Can a trigger be identified? A variety of infections, malignant or autoimmune diseases can contribute to the disturbed immune homeostasis with important consequences for treatment. (iii) Does the patient suffer from a genetic disease predisposing to HLH? Recent advances in the understanding of the genetic and pathophysiological basis of HLH have enabled a better and more rapid answer to this question, which is relevant for prognosis and the decision to perform haematopoietic stem cell transplantation. This review summarizes the current diagnostic approach to the patient with HLH.
AB - Haemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory syndrome characterized by severely disturbed immune homeostasis. It can affect all age groups. Diagnostic evaluation of the patient with suspected HLH has to address three main questions: (i) does the patient have HLH? There is no simple diagnostic test, but a number of clinical and laboratory criteria define this clinical syndrome. (ii) Can a trigger be identified? A variety of infections, malignant or autoimmune diseases can contribute to the disturbed immune homeostasis with important consequences for treatment. (iii) Does the patient suffer from a genetic disease predisposing to HLH? Recent advances in the understanding of the genetic and pathophysiological basis of HLH have enabled a better and more rapid answer to this question, which is relevant for prognosis and the decision to perform haematopoietic stem cell transplantation. This review summarizes the current diagnostic approach to the patient with HLH.
KW - Diagnosis, Differential
KW - Humans
KW - Lymphohistiocytosis, Hemophagocytic/classification/diagnosis/etiology
KW - Diagnosis, Differential
KW - Humans
KW - Lymphohistiocytosis, Hemophagocytic/classification/diagnosis/etiology
M3 - SCORING: Journal article
VL - 160
SP - 275
EP - 287
JO - BRIT J HAEMATOL
JF - BRIT J HAEMATOL
SN - 0007-1048
IS - 3
M1 - 3
ER -
