Hemophagocytic lymphohistiocytosis: a rare diagnosis, an even rarer opportunity to appraise our understanding of the immune system

• Prif Awdur: José Alexandre Marzagão Barbuto (Awdur)
• Fformat: Llyfr
• Cyhoeddwyd: University of São Paulo, 2015-03-01T00:00:00Z.
• Pynciau: article
• Mynediad Ar-lein: Connect to this object online.

The syndrome identified as hemophagocytic lymphohistiocytosis (HLH) poses a rather difficult challenge to the physician. HLH was initially described as a familial disorder,1 which was later associated with various genetic defects that affect the cytotoxic machinery of CD8+ T and NK cells,2 but it al...