Abstract
Fatal graft versus host disease (GVHD) developed in a child with purine nucleoside phosphorylase (PNP) deficiency following an unirradiated platelet transfusion. IV treatment with an anti-T-cell monoclonal antibody (CD7) led to a transient improvement of his GVHD (Grade IV) but did not prevent the fatal outcome. This report emphasizes the need for blood products to be irradiated when cell mediated immunodeficiency is suspected, even in patients with residual immunocompetence.
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Abbreviations
- ADA:
-
adenosine-deaminase
- CD:
-
cluster determinant
- GVHD:
-
graft versus host disease
- PNP:
-
purine nucleoside phosphorylase
- SCID:
-
severe combined immunodeficiency
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Strobel, S., Morgan, G., Simmonds, A.H. et al. Fatal graft versus host disease after platelet transfusions in a child with purine nucleoside phosphorylase deficiency. Eur J Pediatr 148, 312–314 (1989). https://doi.org/10.1007/BF00444121
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DOI: https://doi.org/10.1007/BF00444121