Neutrophil elastase mutations and risk of leukaemia in severe congenital neutropenia
- Authors: Rosenberg, Philip , Alter, Blanche , Link, Daniel , Stein, Steven , Rodger, Elin , Bolyard, Audrey , Aprikyan, Andrew , Bonilla, Mary Ann , Dror, Yigal , Kannourakis, George , Newburger, Peter , Boxer, Laurence , Dale, David
- Date: 2008
- Type: Text , Journal article
- Relation: British Journal of Haematology Vol. 140, no. 2 (2008), p. 210-213
- Full Text: false
- Reviewed:
- Description: Severe congenital neutropenia (SCN) is a heterogeneous bone marrow failure syndrome predisposing to myelodysplastic syndrome and acute myeloid leukaemia (MDS/AML). We studied 82 North American and Australian SCN patients enrolled in the Severe Chronic Neutropenia International Registry who were on long-term treatment with granulocyte colony-stimulating factor and for whom the neutrophil elastase (ELA2) gene was sequenced. There was no significant difference in the risk of MDS/AML in patients with mutant versus wild-type ELA2: the respective cumulative incidences at 15 years were 36% and 25% (P = 0.96). Patients with either mutant or wild-type ELA2 should be followed closely for leukaemic transformation.
- Description: C1
Risk for septic death in severe congenital neutropenia
- Authors: Boxer, Laurence , Bolyard, Audrey , Newburger, Peter , Bonilla, Mary Ann , Kannourakis, George , Dror, Yigal , Link, Daniel , Alter, Blanche , Rosenberg, Philip , Dale, David
- Date: 2008
- Type: Text , Conference paper
- Relation: , p. 1215-1215
- Full Text: false
Cyclic neutropenia is not associated with transformation to MDS and AML
- Authors: Dale, David , Bolyard, Audrey , Newburger, Peter , Bonilla, Mary Ann , Kannourakis, George , Dror, Yigal , Link, Daniel , Alter, Blanche , Rosenberg, Philip , Boxer, Laurence
- Date: 2007
- Type: Text , Journal article
- Relation: Blood Vol. 110, no. 11 (Nov 2007), p. 971A-971A
- Full Text: false
- Reviewed:
- Description: C1
Predictors of transformation to myelodysplasia/acute myelogenous leukemia (MDS/AML) in severe congenital neutropenia (CN)
- Authors: Boxer, Laurence , Bolyard, Audrey , Newburger, Peter , Bonilla, Mary Ann , Kannourakis, George , Dror, Yigal , Link, Daniel , Alter, Blanche , Rosenberg, Philip , Dale, David
- Date: 2007
- Type: Text , Journal article
- Relation: Blood Vol. 110, no. 11 (Nov 2007), p. 971A-971A
- Full Text: false
- Reviewed:
- Description: C1
Severe chronic idiopathic neutropenia in adults : Long-term follow-up of 358 patients
- Authors: Bolyard, Audrey , Pracht, G. , Schwinzer, Beate , Zeidler, Cornelia , Bonilla, Mary Ann , Boxer, Laurence , Cham, Bonnie , Donadieu, J. , Fier, Carol , Freedman, Melvin , Kannourakis, George , Kinsey, Sally , Winkelstein, J. , Alter, Blanche , Reeves, L. , Welte, Karl , Dale, David
- Date: 2003
- Type: Text , Journal article
- Relation: Blood Vol. 102, no. 11 (Nov 2003), p. 273A-273A
- Full Text: false
- Reviewed:
- Description: C1