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Stable long-term risk of leukaemia in patients with severe congenital neutropenia maintained on G-CSF therapy

dc.contributor.authorRosenberg, Philip S.
dc.contributor.authorZeidler, Cornelia
dc.contributor.authorBolyard, Audrey Anna
dc.contributor.authorAlter, Blanche P.
dc.contributor.authorBonilla, Mary A.
dc.contributor.authorBoxer, Laurence A.
dc.contributor.authorDror, Yigal
dc.contributor.authorKinsey, Sally
dc.contributor.authorLink, Daniel C.
dc.contributor.authorNewburger, Peter E.
dc.contributor.authorShimamura, Akiko
dc.contributor.authorWelte, Karl
dc.contributor.authorDale, David C.
dc.date2022-08-11T08:10:10.000
dc.date.accessioned2022-08-23T16:58:16Z
dc.date.available2022-08-23T16:58:16Z
dc.date.issued2010-07-01
dc.date.submitted2012-04-25
dc.identifier.citationBr J Haematol. 2010 Jul;150(2):196-9. Epub 2010 Apr 29. <a href="http://dx.doi.org/10.1111/j.1365-2141.2010.08216.x">Link to article on publisher's website</a>
dc.identifier.issn0007-1048 (Linking)
dc.identifier.doi10.1111/j.1365-2141.2010.08216.x
dc.identifier.pmid20456363
dc.identifier.urihttp://hdl.handle.net/20.500.14038/43329
dc.description.abstractIn severe congenital neutropenia (SCN), long-term therapy with granulocyte colony-stimulating factor (G-CSF) has reduced mortality from sepsis, revealing an underlying predisposition to myelodysplastic syndrome and acute myeloid leukaemia (MDS/AML). We have reported the early pattern of evolution to MDS/AML, but the long-term risk remains uncertain. We updated a prospective study of 374 SCN patients on long-term G-CSF enrolled in the Severe Chronic Neutropenia International Registry. Long-term, the annual risk of MDS/AML attained a plateau (2.3%/year after 10 years). This risk now appears similar to, rather than higher than, the risk of AML in Fanconi anaemia and dyskeratosis congenita.
dc.language.isoen_US
dc.relation<a href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=20456363&dopt=Abstract">Link to article in PubMed</a>
dc.relation.urlhttp://www.ncbi.nlm.nih.gov/pmc/articles/PMC2906693/pdf/nihms-216450.pdf
dc.subjectEpidemiologic Methods
dc.subjectGranulocyte Colony-Stimulating Factor
dc.subjectHumans
dc.subjectLeukemia, Myeloid, Acute
dc.subjectMyelodysplastic Syndromes
dc.subjectNeutropenia
dc.subjectHematology
dc.subjectOncology
dc.subjectPediatrics
dc.titleStable long-term risk of leukaemia in patients with severe congenital neutropenia maintained on G-CSF therapy
dc.typeJournal Article
dc.source.journaltitleBritish journal of haematology
dc.source.volume150
dc.source.issue2
dc.identifier.legacycoverpagehttps://escholarship.umassmed.edu/peds_hematology/131
dc.identifier.contextkey2796622
html.description.abstract<p>In severe congenital neutropenia (SCN), long-term therapy with granulocyte colony-stimulating factor (G-CSF) has reduced mortality from sepsis, revealing an underlying predisposition to myelodysplastic syndrome and acute myeloid leukaemia (MDS/AML). We have reported the early pattern of evolution to MDS/AML, but the long-term risk remains uncertain. We updated a prospective study of 374 SCN patients on long-term G-CSF enrolled in the Severe Chronic Neutropenia International Registry. Long-term, the annual risk of MDS/AML attained a plateau (2.3%/year after 10 years). This risk now appears similar to, rather than higher than, the risk of AML in Fanconi anaemia and dyskeratosis congenita.</p>
dc.identifier.submissionpathpeds_hematology/131
dc.contributor.departmentDepartment of Pediatrics
dc.source.pages196-9


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