Article
Clinical course of patients with major histocompatibility complex
class II deficiency
M A Saleema, P D Arkwrightb, E G Daviesa, A J Cantb, P A Veysa
a The Hospital for
Sick Children, Great Ormond St, London, WC1N 3JH, UK, b Northern Supra-Regional Bone Marrow Transplant
Unit for SCID and Related Disorders, Newcastle General Hospital,
Westgate Rd, Newcastle-upon-Tyne, NE4 6BE, UK
Correspondence to: Dr P D Arkwright, Academic Unit of Child Health, 1st Floor, St Mary's Hospital, Hathersage Rd, Manchester M13 0JH, UK email: mdmfspda{at}fs1.scg.man.ac.uk
Accepted 6 June 2000
The clinical course of 10 children who have been diagnosed with
major histocompatibility complex (MHC) class II deficiency (bare
lymphocyte syndrome) in the UK over the past eight years is described.
They have had a generally poor prognosis, with only two of the 10 still
alive despite eight attempts at bone marrow transplantation in six
patients. Overwhelming viral infection was the predominant cause of
death. Alternative transplant strategies or novel therapies are
required for these patients.
Keywords: bone marrow transplantation; MHC II deficiency; viral infections; outcome
© 2000 by Archives of Disease in Childhood
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