Disseminated BCG infection in severe combined immunodeficiency presenting with severe anaemia and associated with gross hypersplenism after bone marrow transplantation.
An infant with severe combined immunodeficiency (SCID) is described, who presented with severe anaemia and hepatosplenomegaly due to disseminated Bacillus Calmette-Guérin (BCG) infection involving the bone marrow, liver and spleen. After BMT, huge splenic enlargement occurred, presumably due to proliferation of engrafted donor lymphocytes, leading to severe hypersplenism. Peripheral blood cell consumption was resolved by splenectomy, but gradual loss of the marrow graft followed.
Skinner
R
R
Department of Paediatrics, Newcastle General Hospital, Newcastle-upon-Tyne, UK.
Appleton
A L
AL
Sprott
M S
MS
Barer
M R
MR
Magee
J G
JG
Darbyshire
P J
PJ
Abinun
M
M
Cant
A J
AJ
D000740:Anemia / Q000097:blood / Q000209:etiology* / Q000628:therapy; D001500:BCG Vaccine / Q000009:adverse effects; D001772:Blood Cell Count; D016026:Bone Marrow Transplantation / Q000009:adverse effects*; D000075202:Contraindications; D005260:Female; D006085:Graft Survival; D006801:Humans; D006971:Hypersplenism / Q000209:etiology* / Q000601:surgery; D007223:Infant; D009163:Mycobacterium bovis / Q000472:pathogenicity*; D016511:Severe Combined Immunodeficiency / Q000097:blood / Q000150:complications* / Q000628:therapy*; D013156:Splenectomy; D013997:Time Factors; D014184:Transplantation, Homologous; D014376:Tuberculosis / Q000175:diagnosis / Q000209:etiology*
D001500:BCG Vaccine