Skip to main content

Thank you for visiting nature.com. You are using a browser version with limited support for CSS. To obtain the best experience, we recommend you use a more up to date browser (or turn off compatibility mode in Internet Explorer). In the meantime, to ensure continued support, we are displaying the site without styles and JavaScript.

  • Case Report
  • Published:

Successful unrelated bone marrow transplantation for a patient with chronic granulomatous disease and associated resistant pneumonitis and Aspergillus osteomyelitis

Abstract

We describe the successful treatment of a 20-year-old patient with chronic granulomatous disease (CGD), by unrelated bone marrow transplantation (UBMT). The patient is relatively old compared to other CGD patients treated with BMT. He had had repeated serious infections from early childhood and was diagnosed as CGD, gp91-phox deficiency. Prolonged antibiotic-resistant pneumonitis worsened when the patient was 18 years old. In addition, he suffered Aspergillus osteomyelitis and acute renal failure due to amphotericin B. He received 94 granulocyte transfusions from 94 adult donors and the infections gradually improved. In September 1998, at 20 years of age, he underwent UBMT from an HLA 6 antigen-matched male donor, with CY and TBI conditioning. He received MTX and CsA as prophylaxis against GVHD. No serious complications occurred and rapid engraftment was achieved. Acute GVHD (grade 2, at day 19) and chronic GVHD (limited, at day 192) occurred. However, both were easily controlled. The patient is alive and well with no late rejection 26 months after UBMT. Bone Marrow Transplantation (2001) 28, 83–87.

This is a preview of subscription content, access via your institution

Access options

Buy this article

Prices may be subject to local taxes which are calculated during checkout

Figure 1
Figure 2
Figure 3

Similar content being viewed by others

References

  1. The study group of phagocyte disorders of Japan and M Hasui . Chronic granulomatous disease in Japan: incidence and natural history Pediatr Int 1999 41: 589–593

    Article  Google Scholar 

  2. Mouy R, Fischer A, Vilmer E et al. Incidence, severity, and prevention of infections in chronic granulomatous disease J Pediatr 1989 114: 555–560

    Article  CAS  Google Scholar 

  3. Finn A, Hadzic N, Morgan G et al. Prognosis of chronic granulomatous disease Arch Dis Child 1990 65: 942–945

    Article  CAS  Google Scholar 

  4. Winkelstein JA, Marino MC, Johnston RB et al. Chronic granulomatous disease report on a national registry of 368 patients Medicine 2000 79: 155–169

    Article  CAS  Google Scholar 

  5. Di Bartolomeo P, Di Girolamo G, Angrilli F et al. Reconstitution of normal neutrophil function in chronic granulomatous disease by bone marrow transplantation Bone Marrow Transplant 1989 4: 695–700

    CAS  PubMed  Google Scholar 

  6. Zintl F, Hermann J, Fuchs D et al. Correction of fatal genetic diseases using bone marrow transplantation. 2 Kinderarztl Prax 1991 59: 10–15

    CAS  PubMed  Google Scholar 

  7. Hobbs JR, Monteil M, McCluskey DR et al. Chronic granulomatous disease 100% corrected by displacement bone marrow transplantation from a volunteer unrelated donor Eur J Pediatr 1992 151: 806–810

    Article  CAS  Google Scholar 

  8. Ozsahin H, von Planta M, Muller I et al. Successful treatment of invasive aspergillosis in chronic granulomatous disease by bone marrow transplantation, granulocyte colony-stimulating factor-mobilized granulocytes, and liposomal amphotericin B Blood 1998 92: 2719–2724

    CAS  PubMed  Google Scholar 

  9. Ho CM, Vowels MR, Lockwood L et al. Successful bone marrow transplantation in a child with X-linked chronic granulomatous disease Bone Marrow Transplant 1996 18: 213–215

    CAS  Google Scholar 

  10. Calvino MC, Maldonado MS, Otheo E et al. Bone marrow transplantation in chronic granulomatous disease Eur J Pediatr 1996 155: 877–879

    Article  CAS  Google Scholar 

  11. Akioka S, Itoh H, Ueda I et al. Donor lymphocyte infusion at unstable mixed chimerism in an allogenic BMT recipient for chronic granulomatous disease Bone Marrow Transplant 1998 22: 609–611

    Article  CAS  Google Scholar 

  12. Leung TF, Chik KW, Li CK et al. Bone marrow transplantation for chronic granulomatous disease: long term follow-up and review of literature Bone Marrow Transplant 1999 24: 567–570

    Article  CAS  Google Scholar 

  13. Perfect JR, Klotman ME, Gilbert CC et al. Prophylactic intravenous amphotericin B in neutropenic autologous bone marrow transplant recipients J Infect Dis 1992 165: 891–897

    Article  CAS  Google Scholar 

  14. The Westminster Hospitals Bone-Marrow Transplant Team . Bone-marrow transplant from an unrelated donor for chronic granulomatous disease Lancet 1977 1: 210–213

    Google Scholar 

  15. Hobbs JR . Correction of 34 genetic diseases by displacement bone marrow transplantation Plasma Ther Transfus Technol 1985 6: 221–246

    Google Scholar 

  16. Jabado N, Deist FL, Cant A et al. Bone marrow transplantation from genetically HLA-nonidentical donors in children with fatal inherited disorders excluding severe combined immunodeficiencies: use of two monoclonal antibodies to prevent graft Pediatrics 1996 98: 420–428

    CAS  PubMed  Google Scholar 

  17. Fischer A, Landais P, Friedrich W et al. Bone marrow transplantation (BMT) in Europe for primary immunodeficiencies other than severe combined immunodeficiency: a report from the European group for BMT and the European group for immunodeficiency Blood 1994 83: 1149–1154

    CAS  Google Scholar 

Download references

Acknowledgements

We thank Prof Y Kobayashi, Dr S Taniuchi, A Yamamoto, M Hasui and S Tsuji (Dept of Pediatrics, Kansai Medical University) for performing the neutrophil function tests.

Author information

Authors and Affiliations

Authors

Rights and permissions

Reprints and permissions

About this article

Cite this article

Watanabe, C., Yajima, S., Taguchi, T. et al. Successful unrelated bone marrow transplantation for a patient with chronic granulomatous disease and associated resistant pneumonitis and Aspergillus osteomyelitis. Bone Marrow Transplant 28, 83–87 (2001). https://doi.org/10.1038/sj.bmt.1703086

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1038/sj.bmt.1703086

Keywords

This article is cited by

Search

Quick links