The clinical course of 10 children who have been diagnosed with major histo
compatibility 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 infecti
on was the predominant cause of death. Alternative transplant strategies or
novel therapies are required for these patients.