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International Journal of Hematology

, Volume 79, Issue 3, pp 293–297 | Cite as

Fludarabine- and Cyclophosphamide-Based Nonmyeloablative Conditioning Regimen for Transplantation of Chronic Granulomatous Disease: Possible Correlation with Prolonged Pure Red Cell Aplasia

  • Tohru Fujiwara
  • Minami Yamada
  • Koichi Miyamura
  • Yasuo Tomiya
  • Kenichi Ishizawa
  • Hideo Harigae
  • Junichi Kameoka
  • Masayoshi Minegishi
  • Shigeru Tsuchiya
  • Takeshi Sasaki
Case Report

Abstract

An 18-year-old patient with chronic granulomatous disease who had had at least 2 episodes of life-threatening Aspergillus pneumonia was treated with nonmyeloablative allogeneic stem cell transplantation (NSCT) from an HLA-identical and major ABO-incompatible sibling. The conditioning regimen consisted of cyclophosphamide at a dose of 60 mg/kg (days -5, -4) and fludarabine at a dose of 30 mg/m2 (days -5, -4, -3, -2, -1). Full donor T-cell engraftment was attained on day 28, and full myeloid engraftment was established by day 150 after tacrolimus withdrawal.The bacteriocidal activity of neutrophils, as indicated by flow cytometry with the use of a dichlorofluorescein diacetate oxidation assay, remained low until 150 days after transplantation, but no infection was detected, a finding that suggests mixed chimerism of granulocytes controlled infection. Graftversus-host disease and severe regimen-related toxicity (grade 3 or greater) were not observed. This patient developed prolonged pure red cell aplasia, possibly caused by persistent antidonor isohemagglutinin produced by the residual host B-cells. The aplasia resolved with the combination of erythropoietin, double filtration plasmapheresis, and rituximab. In the setting of major ABO-incompatible NSCT, a fludarabine- and cyclophosphamide-based conditioning regimen may lead to prolonged PRCA.

Key words

Chronic granulomatous disease Nonmyeloablative stem cell transplantation Fludarabine- and cyclophosphamide-based conditioning regimen Prolonged pure red cell aplasia 

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Copyright information

© The Japanese Society of Hematology 2004

Authors and Affiliations

  • Tohru Fujiwara
    • 1
  • Minami Yamada
    • 1
  • Koichi Miyamura
    • 1
  • Yasuo Tomiya
    • 1
  • Kenichi Ishizawa
    • 1
  • Hideo Harigae
    • 2
  • Junichi Kameoka
    • 1
  • Masayoshi Minegishi
    • 3
  • Shigeru Tsuchiya
    • 4
  • Takeshi Sasaki
    • 1
  1. 1. Department of Rheumatology and HematologyTohoku University Graduate School of MedicineSendaiJapan
  2. 2.Molecular Diagnostics SendaiJapan
  3. 3.Division of Blood TransfusionSendaiJapan
  4. 4.Department of Pediatric OncologyTohoku University School of MedicineSendaiJapan

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