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Marrow transplantation for Fanconi anaemia: conditioning with reduced doses of cyclophosphamide without radiation.

Abstract：

:Nine patients with Fanconi anaemia (FA) were conditioned for HLA-identical sibling bone marrow transplant (BMT) with reduced dose of cyclophosphamide (Cy) without radiation or antithymocyte globulin (ATG). The total dose of Cy was 140 mg/kg (n = 2) or 120 mg/kg (n = 7). The median patient age was 8 years (range 4-19). Graft-versus-host disease (GVHD) prophylaxis was with methotrexate and cyclosporine (n = 8) or cyclosporine alone (n = 1). All patients had sustained engraftment and two developed grade>/= II acute GVHD. Cy toxicity included grade >/= 2 mucositis seen in all evaluable patients and haemorrhagic cystitis in two patients. The Kaplan-Meler survival estimate is 89% with a median follow-up of 285 d (range 56-528). For the purpose of comparison, this report also reviews and updates long-term follow-up data on 32 previously reported FA patients conditioned with 140-200 mg Cy/kg without radiation. The lowest dose of Cy (without radiation or ATG) after which HLA-identical sibling marrow transplant can be successfully performed in FA patients has yet to be determined, but it appears that uniform and sustained engraftment can be achieved with a Cy dose of as low as 120 mg/kg.

journal_name

Br J Haematol

journal_title

British journal of haematology

authors

Flowers ME,Zanis J,Pasquini R,Deeg HJ,Ribeiro R,Longton G,Medeiros CR,Doney K,Sanders J,Bryant J

doi

10.1046/j.1365-2141.1996.363898.x

subject

Has Abstract,Author List Incomplete

pub_date

1996-03-01 00:00:00

pages

699-706

issue

3

eissn

0007-1048

issn

1365-2141

journal_volume

92

pub_type

杂志文章

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