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Article

Familial hemophagocytic lymphohistiocytosis in two brothers with X-linked agammaglobulinemia.

Pediatric blood & cancer - 1 Aug 2008

Schultz Kris Ann P, Neglia Joseph P, Smith Angela R, Ochs Hans D, Torgerson Troy R, Kumar Ashish

Abstract excerpt

Hemophagocytic lymphohistiocytosis (HLH) is often familial and is associated with high mortality. Primary (familial) HLH is known to occur in children with mutations in perforin, Munc13-4, or syntaxin 11. We describe a case series of two brothers who developed HLH in the setting of X-linked agammaglobulinemia (XLA, Bruton's disease) and adenovirus infection. Further studies revealed absence of Bruton's tyrosine...

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Identifiers

PMID: 18421721
DOI: 10.1002/pbc.21573

Genes

BTK

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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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