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Article

Subtle differences in CTL cytotoxicity determine susceptibility to hemophagocytic lymphohistiocytosis in mice and humans with Chediak-Higashi syndrome.

Blood - 27 Oct 2011

Jessen Birthe, Maul-Pavicic Andrea, Ufheil Heike, Vraetz Thomas, Enders Anselm, Lehmberg Kai, Längler Alfred, Gross-Wieltsch Ute, Bay Ali, Kaya Zuhre, Bryceson Yenan T, Koscielniak Ewa, Badawy Sherif, Davies Graham, Hufnagel Markus, Schmitt-Graeff Annette, Aichele Peter, Zur Stadt Udo, Schwarz Klaus, Ehl Stephan

Abstract excerpt

Perforin-mediated cytotoxicity is important for controlling viral infections, but also for limiting immune reactions. Failure of this cytotoxic pathway leads to hemophagocytic lymphohistiocytosis (HLH), a life-threatening disorder of uncontrolled T-cell and macrophage activation. We studied susceptibility to HLH in 2 mouse strains (souris and beige(J)) and a cohort of patients with partial defects in perforin...

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PMID: 21878672
DOI: 10.1182/blood-2011-05-356113

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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