徳島大学 教育・研究者情報データベース(EDB)

1. (英) Nishi Masanori

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2. (英) Nishimura Ryosei

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3. (英) Suzuki Nobuhiro

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4. (英) Sawada Akihisa

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5. (英) Okamura Takayuki

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6. (英) Fujita Naoto

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7. (英) Kanai Rie

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8. (英) Yano Jun

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9. (英) Adachi Souichi

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10. (英) Yasumi Takahiro

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11. (英) Sato Emiko

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12. 安友 康二([徳島大学.大学院医歯薬学研究部.医学域.医科学部門.病理系.生体防御医学])

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13. (英) Ishii Eiichi

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14. (英) Ohga Shouichi

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(英) Reduced-intensity conditioning in unrelated donor cord blood transplantation for familial hemophagocytic lymphohistiocytosis.

(英) Familial hemophagocytic lymphohistiocytosis (FHL) is a disorder of immune homeostasis characterized by fever, cytopenias, hepatosplenomegaly, and coagulopathy. We studied the outcomes of 13 FHL patients who underwent the first unrelated cord blood transplantation (UCBT) after non-myeloablative conditionings. The major regimen consisted of fludarabine (FLU; n = 12)+melphalan (MEL; n = 11)± low-dose total body irradiation (TBI 2-4 Gy; n = 6). The median age at presentation and period to UCBT were 6 and 5 months, respectively. Central nervous system (CNS) disease developed in one infant at diagnosis, and in two others until UCBT. HLH activity was controlled in all but one at the time of UCBT. Ten patients had early engraftment on median day 21 with no grade >2 treatment-related toxicity and two controllable grade >2 acute GVHD. Two patients with early rejection successfully underwent subsequent UCBT after myeloablative conditioning. Two others had late graft failure following mixed donor chimerism. Two deaths occurred from HLH; early liver failure and late CNS disease. Of 11 FLU+MEL-conditioned patients, the frequency of disease-free complete engraftment was higher for MEL (120 mg/m(2) )+TBI, or high-dose MEL (180 mg/m(2) ) than for others (83% vs. 25%, p = 0.036). The FLU+MEL-based non-myeloablative regimen was acceptable for FHL infants undergoing UCBT, although further studies will be needed for confirmation.

1. (英) Blood Component Transfusion
2. (英) Blood Donors
3. (英) Child, Preschool
4. (英) Combined Modality Therapy
5. (英) Cord Blood Stem Cell Transplantation
6. (英) Disease-Free Survival
7. (英) Female
8. (英) Graft Survival
9. (英) Graft vs Host Disease
10. (英) Humans
11. (英) Infant
12. (英) Infant, Newborn
13. (英) Japan
14. (英) Lymphohistiocytosis, Hemophagocytic
15. (英) Male
16. (英) Melphalan
17. (英) Reoperation
18. (英) Retrospective Studies
19. (英) Salvage Therapy
20. (英) Transplantation Conditioning
21. (英) Treatment Outcome
22. (英) Vidarabine
23. (英) Whole-Body Irradiation

1. (英) Affiliation: Department of Pediatrics, Saga University Hospital, Saga, Japan.

2. (英) PublicationType: Evaluation Studies

3. (英) PublicationType: Letter