Clinical course of patients with major histocompatibility complex class II deficiency

M A Saleem; P D Arkwright; E G Davies; A J Cant; P A Veys

doi:10.1136/adc.83.4.356

Clinical course of patients with major histocompatibility complex class II deficiency

Arch Dis Child. 2000 Oct;83(4):356-9. doi: 10.1136/adc.83.4.356.

Authors

M A Saleem¹, P D Arkwright, E G Davies, A J Cant, P A Veys

Affiliation

¹ The Hospital for Sick Children, Great Ormond St, London, WC1N 3JH, UK.

Abstract

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.

MeSH terms

Bone Marrow Transplantation
Female
Follow-Up Studies
Histocompatibility Antigens Class II / blood*
Humans
Infant
Infant, Newborn
Male
Opportunistic Infections / complications*
Opportunistic Infections / immunology
Prognosis
Severe Combined Immunodeficiency / complications*
Severe Combined Immunodeficiency / immunology
Severe Combined Immunodeficiency / therapy
Survival Rate
Virus Diseases / complications
Virus Diseases / immunology

Substances

Histocompatibility Antigens Class II