Is an infectious trigger always required for primary hemophagocytic lymphohistiocytosis? Lessons from in utero and neonatal disease
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Is an infectious trigger always required for primary hemophagocytic lymphohistiocytosis? Lessons from in utero and neonatal disease. / Heeg, Maximilian; Ammann, Sandra; Klemann, Christian; Panning, Marcus; Falcone, Valeria; Hengel, Harmut; Lehmberg, Kai; Zur Stadt, Udo; Wustrau, Katharina; Janka, Gritta; Ehl, Stephan.
In: PEDIATR BLOOD CANCER, Vol. 65, No. 11, 01.08.2018, p. e27344.Research output: SCORING: Contribution to journal › SCORING: Journal article › Research › peer-review
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TY - JOUR
T1 - Is an infectious trigger always required for primary hemophagocytic lymphohistiocytosis? Lessons from in utero and neonatal disease
AU - Heeg, Maximilian
AU - Ammann, Sandra
AU - Klemann, Christian
AU - Panning, Marcus
AU - Falcone, Valeria
AU - Hengel, Harmut
AU - Lehmberg, Kai
AU - Zur Stadt, Udo
AU - Wustrau, Katharina
AU - Janka, Gritta
AU - Ehl, Stephan
N1 - © 2018 Wiley Periodicals, Inc.
PY - 2018/8/1
Y1 - 2018/8/1
N2 - In this report, we evaluate the hypothesis that hemophagocytic lymphohistiocytosis in patients with defects of lymphocyte cytotoxicity is usually triggered by infections. We show that in the majority of patients, extensive virus PCR panels performed in addition to routine microbiological investigations remain negative and summarize 25 patients with onset of hemophagocytic lymphohistiocytosis in utero or within the first 10 days of life, in none of which an associated bacterial or viral infection was reported. These observations, even though preliminary, invite to consider a key role of lymphocyte cytotoxicity in controlling T-cell homeostasis also in the absence of apparent infectious stimuli.
AB - In this report, we evaluate the hypothesis that hemophagocytic lymphohistiocytosis in patients with defects of lymphocyte cytotoxicity is usually triggered by infections. We show that in the majority of patients, extensive virus PCR panels performed in addition to routine microbiological investigations remain negative and summarize 25 patients with onset of hemophagocytic lymphohistiocytosis in utero or within the first 10 days of life, in none of which an associated bacterial or viral infection was reported. These observations, even though preliminary, invite to consider a key role of lymphocyte cytotoxicity in controlling T-cell homeostasis also in the absence of apparent infectious stimuli.
KW - Journal Article
U2 - 10.1002/pbc.27344
DO - 10.1002/pbc.27344
M3 - SCORING: Journal article
C2 - 30070073
VL - 65
SP - e27344
JO - PEDIATR BLOOD CANCER
JF - PEDIATR BLOOD CANCER
SN - 1545-5009
IS - 11
ER -