TY - JOUR
T1 - Successful umbilical cord blood transplantation from an unrelated donor for a patient with Epstein-Barr virus-associated hemophagocytic lymphohistiocytosis
AU - Minegishi, M.
AU - Ohashi, Y.
AU - Kumaki, S.
AU - Sasahara, Y.
AU - Hayashi, T.
AU - Asada, H.
AU - Okuyama, T.
AU - Hakozaki, I.
AU - Sato, T.
AU - Tsuchiya, S.
N1 - Funding Information:
We are indebted to the Tokai Cord Blood Bank for supplying the cord blood stem cells. We are also grateful to Dr Hiroshi Kimura (Nagoya University School of Medicine, Nagoya, Japan) for providing the BALF5 gene construct. This work was supported in part by a Grant-in-Aid for Scientific Research from the Ministry of Education, Science, Sports and Culture, Japan.
PY - 2001
Y1 - 2001
N2 - We report a case of a 5-year-old girl with EBV-associated hemophagocytic lymphohistiocytosis (EBV-HLH) who underwent cord blood (CB) stem cell transplantation (CBSCT) from an unrelated donor. The patient presented with persistent high-grade fever and hepatosplenomegaly. Because the disease was refractory to immunochemotherapy according to the HLH94 protocol, she received 2.0 × 107 CB nucleated cells/kg body weight (BW) after conditioning with BU/CY/etoposide. No acute GVHD developed, using FK506 for prophylaxis. The neutrophil count reached >0.5 × 109/l by day 21 and the platelet count reached >50 × 109/l by day 84. The patient recovered well with sequelae of neurological deficits more than 10 months after receiving CBSCT, without showing evidence of HLH or chronic GVHD. Real-time PCR proved applicable for estimation of the EBV load in PBMC of the patient. We conclude that CBSCT may be indicated for some cases of refractory EBV-HLH, who have no HLA-matched siblings and are therefore dependent on unrelated marrow donors.
AB - We report a case of a 5-year-old girl with EBV-associated hemophagocytic lymphohistiocytosis (EBV-HLH) who underwent cord blood (CB) stem cell transplantation (CBSCT) from an unrelated donor. The patient presented with persistent high-grade fever and hepatosplenomegaly. Because the disease was refractory to immunochemotherapy according to the HLH94 protocol, she received 2.0 × 107 CB nucleated cells/kg body weight (BW) after conditioning with BU/CY/etoposide. No acute GVHD developed, using FK506 for prophylaxis. The neutrophil count reached >0.5 × 109/l by day 21 and the platelet count reached >50 × 109/l by day 84. The patient recovered well with sequelae of neurological deficits more than 10 months after receiving CBSCT, without showing evidence of HLH or chronic GVHD. Real-time PCR proved applicable for estimation of the EBV load in PBMC of the patient. We conclude that CBSCT may be indicated for some cases of refractory EBV-HLH, who have no HLA-matched siblings and are therefore dependent on unrelated marrow donors.
KW - Allogeneic transplantation
KW - EBV
KW - Hemophagocytic lymphohistiocytosis
KW - Real-time PCR
KW - Umbilical cord blood stem cell
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U2 - 10.1038/sj.bmt.1702999
DO - 10.1038/sj.bmt.1702999
M3 - Article
C2 - 11477448
AN - SCOPUS:17844369430
SN - 0268-3369
VL - 27
SP - 883
EP - 886
JO - Bone Marrow Transplantation
JF - Bone Marrow Transplantation
IS - 8
ER -