NCBI: db=pubmed; Term=haemophagocytic lymphohistiocytosis
Janus kinase inhibition for autoinflammation in DNASE2 deficiency.
J Allergy Clin Immunol. 2019 Nov 24;:
Authors: Hong Y, Capitani M, Murphy C, Pandey S, Cavounidis A, Takeshita H, Nanthapisal S, Yasuda T, Bader-Meunier B, McCreary D, Omoyinmi E, Rao A, Booth C, Gilmour K, Sebire N, Shah N, Klein N, Bullock AN, Eleftheriou D, Uhlig HH, Brogan P
CAPSULE SUMMARY: We characterise the immunopathogenesis of autoinflammation, HLH, and early onset intestinal inflammation caused by homozygous mutation in DNASE2, and provide the first description of favourable therapeutic response to JAK1/JAK2 blockade in this disease.
PMID: 31775019 [PubMed – as supplied by publisher]