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

Toyoda, H; Azuma, E; Hori, H; Hirayama, M; Kobayashi, M; Isogai, K; Kondo, N; Komada, Y

Kostmann syndrome, severe congenital neutropenia, is often associated with life-threatening bacterial infections. A 5-year-old girl with Kostmann syndrome developed pulmonary abscesses. She was refractory to granulocyte colony-stimulating factor and antibiotics. She underwent unrelated HLA-matched BMT. Myeloablative conditioning consisted of 12-Gy TBI with lung shielding, antithymocyte globulin, etoposide, and cyclophosphamide. After successful engraftment, the pulmonary abscesses resolved by day 75 post-transplant. Although the option of transplantation is not established in the setting of unrelated 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. Bone Marrow Transplantation (2001) 28, 413–415.

LUNG abscesses; BONE marrow transplantation; SYNDROMES; THERAPEUTICS

Bone Marrow Transplantation, 2001, Vol 28, Issue 4, p413

0268-3369

Academic Journal

10.1038/sj.bmt.1703156