title: Donor leukocyte infusion after hematopoietic stem cell transplantation in patients with juvenile myelomonocytic leukemia.
abstract: Juvenile myelomonocytic leukemia (JMML) is a clonal myeloproliferative disorder of early childhood. In all, 21 patients with JMML who received donor leukocyte infusion (DLI) after allogeneic hematopoietic stem cell transplantation (HSCT) for either mixed chimerism (MC, n=7) or relapse (n=14) were studied. Six patients had been transplanted from an HLA-matched sibling and 15 from other donors. Six of the 21 patients (MC: 3/7 patients; relapse: 3/14 patients) responded to DLI. Response rate was significantly higher in patients receiving a higher total T-cell dose (> or =1 x 10(7)/kg) and in patients with an abnormal karyotype. None of the six patients receiving DLI from a matched sibling responded. Response was observed in five of six patients who did and in one of 15 children who did not develop acute graft-versus-host disease following DLI (P=0.01). The overall outcome was poor even for the responders. Only one of the responders is alive in remission, two relapsed, and three died of complications. In conclusion, this study shows that some cases of JMML may be sensitive to DLI, this providing evidence for a graft-versus-leukemia effect in JMML. Infusion of a high number of T cells, strategies to reduce toxicity, and cytoreduction prior to DLI may improve the results.
authors.affiliation: Department of Pediatrics and Adolescent Medicine, Division of Pediatric Hematology and Oncology, University of Freiburg, Freiburg 79106, Germany. yoshimi@kikli.ukl.uni-freiburg.de
authors.forename: A
authors.initials: A
authors.lastname: Yoshimi
authors.forename: P
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authors.lastname: Bader
authors.forename: S
authors.initials: S
authors.lastname: Matthes-Martin
authors.forename: J
authors.initials: J
authors.lastname: Starý
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authors.lastname: Sedlacek
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authors.lastname: Duffner
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authors.lastname: Klingebiel
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authors.lastname: Dilloo
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authors.lastname: Holter
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authors.lastname: Zintl
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authors.lastname: Kremens
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authors.lastname: Sykora
authors.forename: C
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authors.lastname: Urban
authors.forename: H
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authors.lastname: Hasle
authors.forename: E
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authors.lastname: Korthof
authors.forename: T
authors.initials: T
authors.lastname: Révész
authors.forename: A
authors.initials: A
authors.lastname: Fischer
authors.forename: P
authors.initials: P
authors.lastname: Nöllke
authors.forename: F
authors.initials: F
authors.lastname: Locatelli
authors.forename: C M
authors.initials: CM
authors.lastname: Niemeyer
mesh_terms: D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D005260:Female; D005500:Follow-Up Studies; D006086:Graft vs Host Disease / Q000209:etiology; D018380:Hematopoietic Stem Cell Transplantation / Q000009:adverse effects* / Q000401:mortality; D006801:Humans; D007223:Infant; D015477:Leukemia, Myelomonocytic, Chronic / Q000401:mortality / Q000628:therapy*; D017708:Leukocyte Transfusion; D008297:Male; D012008:Recurrence; D018183:Transplantation Chimera; D014184:Transplantation, Homologous; D016896:Treatment Outcome