Successful unrelated BMT in a patient with Kostmann syndrome complicated by pre-transplant pulmonary 'bacterial' abscesses

Kostmann syndrome, severe congenital neutropenia, is often associated with life-threatening bacterial infections. A 5-year-old girl with Kostmann synd rome developed pulmonary abscesses. She was refractory to granulocyte colon y-stimulating factor and antibiotics. She underwent unrelated HLA-matched B MT. Myeloablative conditioning consisted of 12-Gy TBI with lung shielding, antithymocyte globulin, etoposide, and cyclophosphamide. After successful e ngraftment, the pulmonary abscesses resolved by day 75 post-transplant. Alt hough the option of transplantation is not established in the setting of un related HLA-matched BMT in Kostmann syndrome, this case may provide useful information. Furthermore, pre-transplant pulmonary bacterial abscesses may not be a contraindication for BMT in some patients with Kostmann syndrome.