Abstract
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.
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References
Kostman R . Infantile genetic agranulocytosis. A review with presentation of ten new cases Acta Paediatr Scand 1975 64: 362–368
Bonilla MA, Gillio AP, Ruggeiro M et al. Effects of recombinant human granulocyte colony-stimulating factor on neutropenia in patients with congenital agranulocytosis New Engl J Med 1989 320: 1574–1580
Welte K, Boxer LA . Severe chronic neutropenia: pathophysiology and therapy Semin Hematol 1997 34: 267–278
Zeidler C, Welte K, Barak Y et al. Stem cell transplantation in patients with severe congenital neutropenia without evidence of leukemic transformation Blood 2000 95: 1195–1198
Obayashi T, Yoshida M, Mori T et al. Plasma (1–>3)-beta-D-glucan measurement in diagnosis of invasive deep mycosis and fungal febrile episodes Lancet 1995 345: 17–20
Ishikawa N, Suzuki H, Sato T et al. Effectiveness of Pastorex Aspergillus for diagnosis of invasive Aspergillosis Clin Microbiol 1992 19: 241–246 (in Japanese)
Aihara M, Sawada Y, Takami H et al. Cand-Tec test for detection of Candida antigen J Med Technol 1990 34: 367–370 (in Japanese)
Kudo T, Kaminuma Y . Serological reactions to pulmonary Aspergillosis Jap J Clin Med 1980 38: 4354–4361 (in Japanese)
Motoyoshi K . Biological activities and clinical application of M-CSF Int J Hematol 1998 67: 109–122
Ruutu P, Valtonen V, Elonen E et al. Invasive pulmonary aspergillosis: a diagnostic and therapeutic problem. Clinical experience with eight haematologic patients Scand J Infect Dis 1987 19: 569–575
Cafferkey MT, Abrahamson E, Bloom A, Keane CT . Pulmonary infection due to methicillin-resistant Staphylococcus aureus Scand J Infect Dis 1988 20: 297–301
Abu Shara KA, Ghareeb MA, Zaher S et al. Radiotherapeutic effect on oropharyngeal flora in patients with head and neck cancer J Laryngol Otol 1993 107: 222–227
Hoover M, Morgan ER, Kletzel M . Prior fungal infection is not a contraindication to bone marrow transplant in patients with acute leukemia Med Pediatr Oncol 1997 28: 268–273
Agematsu K, Nakahata T, Kitahara H et al. Successful bone marrow transplantation in a patient with severe pretransplant pneumonia Transplantation 1990 50: 326–327
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Toyoda, H., Azuma, E., Hori, H. et al. Successful unrelated BMT in a patient with Kostmann syndrome complicated by pre-transplant pulmonary ‘bacterial’ abscesses. Bone Marrow Transplant 28, 413–415 (2001). https://doi.org/10.1038/sj.bmt.1703156
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DOI: https://doi.org/10.1038/sj.bmt.1703156
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