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Article

Prolonged course of familial hemophagocytic lymphohistiocytosis.

Journal of pediatric hematology/oncology - 1 Dec 2006

Steinberg Orna, Yacobovich Joanne, Dgany Orly, Kodman Yona, Livni Gilat, Rachmel Avinoam, Stein Jerry, Yaniv Isaac, Tamary Hannah

Abstract excerpt

Familial hemophagocytic lymphohistiocytosis is usually diagnosed in the first 2 years of life and, if untreated, is rapidly fatal. We describe a 10-year-old boy with a 9-year history of prolonged fever and progressive hepatosplenomegaly who was diagnosed as having hemophagocytic lymphohistiocytosis 2, being homozygote to a previously described mutation in the PRF1 gene, and cured by the HLH-2004 protocol and...

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Source

Identifiers

PMID: 17164654
DOI: 10.1097/MPH.0b013e31802d3a96

Genes

PRF1

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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