Allogenic Bone Marrow Transplantation for Congenital Neutropenia: A Case Report.
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Although recombinant granulocyte colony stimulating factor (rG-CSF) improves a prognosis of congenital neutropenia patients, we report here a 5-year-old female congenital neutropenia who did not respond to rG-CSF therapy and received allogeneic bone marrow transplantation. Bone marrow cells were transplanted from her HLA-matched sister following a thoracoabdominal irradiation and cyclophosphamide preconditioning therapy. Her peripheral blood neutrophils reached 500/μl on day 19. Complete blood counts were normal and stable, and also a frequency of bacterial infection was markedly reduced. However, hemopoietic cells showed mixed chimerism on day 244 and thereafter. On day 540 she showed pancytopenia unexpectedly and was suspected of a late phase marrow rejection. Despite the neutrophil response to high dose rG-CSF, donor hemopoiesis did not recover. The second transplant was performed from the same donor and with conditioning regimen which included cyclophosphamide with total body plus total lymph node irradiation. Donor cells were rejected again and she died on day 51 of the second transplant. It is discussed that a preconditioning therapy for these patients should be intensified with other immunosupressants.
- 特定非営利活動法人 日本小児血液・がん学会の論文
特定非営利活動法人 日本小児血液・がん学会 | 論文
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