An unconditioned bone marrow transplantation in a child with purine nucleoside phosphorylase deficiency and its unique complication

Caner Aytekin, Mutlu Yuksek, Figen Dogu, Aydin Yagmurlu, Alisan Yildiran, Suat Fitoz, Meltem Kologlu, Emel Babacan, Michael S. Hershfield, Aydan Ikinciogullari

doi:10.1111/j.1399-3046.2007.00890.x

An unconditioned bone marrow transplantation in a child with purine nucleoside phosphorylase deficiency and its unique complication

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説明

<jats:p><jats:bold>Abstract: </jats:bold> Purine nucleoside phosphorylase deficiency is a rare immunodeficiency syndrome characterized by recurrent infections, neurological dysfunction, and autoimmunity. Early diagnosis and hematopoietic stem cell transplantation may reverse the dismal prognosis in PNP deficiency. This report presents a new PNP deficiency case successfully transplanted without a conditioning regimen from an HLA‐identical family donor, who developed a complication of disseminated BCG infection.</jats:p>