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Clinical course of patients with major histocompatibility complex class II deficiency
  1. M A Saleema,
  2. P D Arkwrightb,
  3. E G Daviesa,
  4. A J Cantb,
  5. P A Veysa
  1. aThe Hospital for Sick Children, Great Ormond St, London, WC1N 3JH, UK, bNorthern Supra-Regional Bone Marrow Transplant Unit for SCID and Related Disorders, Newcastle General Hospital, Westgate Rd, Newcastle-upon-Tyne, NE4 6BE, UK
  1. Dr P D Arkwright, Academic Unit of Child Health, 1st Floor, St Mary's Hospital, Hathersage Rd, Manchester M13 0JH, UK email:mdmfspda{at}


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.

  • bone marrow transplantation
  • MHC II deficiency
  • viral infections
  • outcome

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