European Journal of Pediatrics

, Volume 150, Issue 7, pp 477–480 | Cite as

Successful bone marrow transplantation and treatment of BCG infection in two patients with severe combined immunodeficiency

  • R. S. Heyderman
  • G. Morgan
  • R. J. Levinsky
  • S. Strobel


We report successful bone marrow transplantation (BMT) in two patients with severe combined immunodeficiency (SCID), who had developed BCG infection following neonatal vaccination. Patient 1 had Omenn Syndrome, associated with hypertrophic nonobstructive cardiomyopathy. Patient 2 had SCID due to adenosine deaminase deficiency. This communication demonstrates that with appropriate anti-mycobacterial cover, immunological reconstitution together with full recovery from BCG infection can be achieved by BMT. As demonstrated by persistant negative Mantoux tests, specific cell-mediated immunity to BCG was not acquired following BMT. We suggest that these children may continue to be at risk from mycobacterial infection.

Key words

BCG infection Severe combined immunodeficiency Omenn syndrome Bone marrow transplantation Hypertrophic non-obstructive cardiomyopathy 



bone marrow transplantation


hypertrophic nonobstructive cardiomyopathy


severe combined immunodeficiency


Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.


  1. 1.
    Alfaham MA, Ferguson SD, Shira B, Davies J (1987) The idiopathic hypereosinophilic syndrome. Arch Dis Child 62: 601–613PubMedGoogle Scholar
  2. 2.
    Genin G, Touraine J-L, Berger E, Bryon P-A, Valancogne A, Phillipe N, Monet P (1977) BCGite generalisée dans und déficit immunitaire mixte et grave. Evolution defavourable malgre une tentative de greffe moelle osseuse. Arch Fr Pediatr 34: 639–648PubMedGoogle Scholar
  3. 3.
    Gonzalez B, Moreno S, Burdach R, Valenzuela MT, Henriquez A, Ramos MI, Sorensen RU (1989) Clinical presentation of Bacillus Calmette-Guérin infections in patients with immunodeficiency syndromes. Pediatr Infect Dis J 8:201–206PubMedGoogle Scholar
  4. 4.
    Jouan H, Le-Deist F, Nezelof C (1987) Omenn's syndrome — pathologic arguments in favour of graft versus host pathogenesis: a report of nine cases. Hum Pathol 18:1101–1108PubMedGoogle Scholar
  5. 5.
    Junker AK, Chan KW, Massing BG (1989) Clinical and immune recovery from Omenn Syndrome after bone marrow transplantation. J Pediatr 114:596–600PubMedGoogle Scholar
  6. 6.
    Katz F, Malcolm S, Strobel S, Finn A, Morgan G, Levinsky RJ (1990) The use of locus-specific minisatellite probes to check engraftment following bone marrow transplantation for severe combined immunodeficiency disease. Bone Marrow Transplant 5:199–204PubMedGoogle Scholar
  7. 7.
    Minegishi M, Tsuchiya S, Imaizumi M, Yamaguchi Y, Goto Y, Tamura M, Kono T, Tada K (1985) Successful transplantation of soy bean agglutinin-fractionated, histocompatible, maternal marrow in a patient with severe combined immunodeficiency and BCG infection. Eur J Pediatr 143:291–294CrossRefPubMedGoogle Scholar
  8. 8.
    Morgan G, Levinsky RJ, Hugh-Jones K, Fairbanks LD, Morris GS, Simmonds HA (1987) Heterogeneity of biochemical, clinical and immunological parameters in severe combined immunodeficiency due to adenosine deaminase deficiency. Clin Exp Immunol 70:491–499PubMedGoogle Scholar
  9. 9.
    Omenn GS (1965) Familial Reticuloendotheliosis with cosinophilia. N Engl J Med 273:427–432PubMedGoogle Scholar
  10. 10.
    Quinn TC (1989) Interactions of the immunodeficiency virus and tuberculosis and the implications for BCG vaccination. Rev Infect Dis 11: S 379–384PubMedGoogle Scholar
  11. 11.
    Scoazec LY, Fischer A, Nezelof C (1984) Les infections généralisées à BCG, expression d'un deficit multi-factoriel de la bactericidie intramacrophagique: étude anatomo-clinique de 11 cas. Arch Fr Pediatr 41:681–687PubMedGoogle Scholar
  12. 12.
    Tachibana N, Okayama A, Ishizaki J, Yokota T, Shishime E, Murai K, Shioiri S, Tsuda K, Essex M, Mueller N (1988) Suppression of tuberculin skin reaction in healthy HTLV-1 carriers from Japan. Int J Cancer 42:829–831PubMedGoogle Scholar

Copyright information

© Springer-Verlag 1991

Authors and Affiliations

  • R. S. Heyderman
    • 1
    • 2
  • G. Morgan
    • 1
    • 2
  • R. J. Levinsky
    • 1
    • 2
  • S. Strobel
    • 1
    • 2
  1. 1.Department of ImmunologyInstitute of Child HealthLondonUK
  2. 2.Hospital for Sick ChildrenLondonUK

Personalised recommendations