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.

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@article{7aa6e448d5124a06845f102858039a1e,
title = "Is an infectious trigger always required for primary hemophagocytic lymphohistiocytosis? Lessons from in utero and neonatal disease",
abstract = "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.",
keywords = "Journal Article",
author = "Maximilian Heeg and Sandra Ammann and Christian Klemann and Marcus Panning and Valeria Falcone and Harmut Hengel and Kai Lehmberg and {Zur Stadt}, Udo and Katharina Wustrau and Gritta Janka and Stephan Ehl",
note = "{\textcopyright} 2018 Wiley Periodicals, Inc.",
year = "2018",
month = aug,
day = "1",
doi = "10.1002/pbc.27344",
language = "English",
volume = "65",
pages = "e27344",
journal = "PEDIATR BLOOD CANCER",
issn = "1545-5009",
publisher = "Wiley-Liss Inc.",
number = "11",

}

RIS

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 -