Acquired autoimmune thrombocytopenia post-bone marrow transplantation for severe combined immunodeficiency.
Children with severe combined immunodeficiency (SCID) have profoundly diminished humoral and cellular immunity resulting in death during infancy unless immune reconstitution occurs by bone marrow transplantation (BMT). Thrombocytopenia post-bone marrow transplantation can be seen in relation to infection, graft-versus-host disease (GVHD) and rarely, as an autoimmune phenomenon due to immune dysregulation. We report two cases of severe AITP following BMT for SCID. Both cases developed large intracerebral hemorrhages from which one died. Autoimmune thrombocytopenia in this setting can be life-threatening and we recommend early and active intervention.
Ting
S S
SS
Department of Immunology/Allergy, Sydney Children's Hospital, NSW, Australia.
Ziegler
J B
JB
Vowels
M R
MR
D016026:Bone Marrow Transplantation / Q000009:adverse effects*; D005260:Female; D006801:Humans; D007223:Infant; D016553:Purpura, Thrombocytopenic, Idiopathic / Q000209:etiology*; D016511:Severe Combined Immunodeficiency / Q000628:therapy*