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Blood, 15 June 2006, Vol. 107, No. 12, pp. 4628-4635. Prepublished online as a Blood First Edition Paper on February 23, 2006; DOI 10.1182/blood-2005-11-4370. This Article Full Text Full Text (PDF) All Versions of this Article: 2005-11-4370v1 107/12/4628    most recent Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Rights and Permissions Citing Articles Citing Articles via HighWire Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Rosenberg, P. S. Articles by Dale, D. C. Search for Related Content PubMed PubMed Citation Articles by Rosenberg, P. S. Articles by Dale, D. C. Related Collections Neoplasia Phagocytes Chemokines, Cytokines, and Interleukins Clinical Trials and Observations Related Article in Blood Online Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  CLINICAL TRIALS AND OBSERVATIONS The incidence of leukemia and mortality from sepsis in patients with severe congenital neutropenia receiving long-term G-CSF therapy Philip S. Rosenberg, Blanche P. Alter, Audrey A. Bolyard, Mary Ann Bonilla, Laurence A. Boxer, Bonnie Cham, Carol Fier, Melvin Freedman, George Kannourakis, Sally Kinsey, Beate Schwinzer, Connie Zeidler, Karl Welte, David C. Dale, for the Severe Chronic Neutropenia International Registry From the Biostatistics Branch and Clinical Genetics Branch, Division of Cancer Epidemiology and Genetics, National Cancer Institute, National Institutes of Health, Department of Health and Human Services, Rockville, MD; Department of Medicine, University of Washington, Seattle, WA; Department of Pediatric Hematology Oncology, St Joseph's Children's Hospital, Paterson, NJ; Department of Pediatrics, University of Michigan Medical Center, Ann Arbor, MI; CancerCare Manitoba, Winnipeg, MB, Canada; Amgen, Inc, Boulder, CO; Hospital for Sick Children, Toronto, ON, Canada; Ballarat Oncology and Haemotology Services, Wendouree, Australia; St James's University Hospital, Leeds, United Kingdom; and Kinderklinik, Medizinische Hochschule, Hannover, Germany. In patients with severe congenital neutropenia (SCN), sepsis mortality is reduced by treatment with granulocyte colony-stimulating factor (G-CSF), but myelodsyplastic syndrome and acute myeloid leukemia (MDS/AML) have been reported. We studied 374 patients with SCN and 29 patients with Shwachman-Diamond syndrome (SDS) on long-term G-CSF enrolled in the Severe Chronic Neutropenia International Registry. In SCN, sepsis mortality was stable at 0.9% per year. The hazard of MDS/AML increased significantly over time, from 2.9% per year after 6 years to 8.0% per year after 12 years on G-CSF. After 10 years, the cumulative incidence was 8% for sepsis mortality and 21% for MDS/AML. A subgroup of SCN patients (29%) received more than the median dose of G-CSF ( 8 µg/kg/d), but achieved less than the median absolute neutrophil count (ANC) response (ANC < 2.188 x 109/L [2188/µL] at 6-18 months). In these less-responsive patients, the cumulative incidence of adverse events was highest: after 10 years, 40% developed MDS/AML and 14% died of sepsis, compared with 11% and 4%, respectively, of more responsive patients whose ANC was above the median on doses of G-CSF below the median. Risk of MDS/AML may be similar in SDS and SCN. In less-responsive SCN patients, early hematopoietic stem cell transplantation may be a rational option. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? Related Article in Blood Online: Reconsidering how we treat severe congenital neutropenia Kevin Shannon Blood 2006 107: 4575-4576. [Full Text] [PDF] This article has been cited by other articles: K. Beel and P. Vandenberghe G-CSF receptor (CSF3R) mutations in X-linked neutropenia evolving to acute myeloid leukemia or myelodysplasia Haematologica, October 1, 2009; 94(10): 1449 - 1452. [Abstract] [Full Text] [PDF] M. Czader and A. 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