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
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
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
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
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
Severe chronic neutropenia : Treatment and follow-up of patients in the severe chronic neutropenia international registry
- Authors: Dale, David , Cottle, Tammy , Fier, Carol , Bolyard, Audrey , Bonilla, Mary Ann , Boxer, Laurence , Cham, Bonnie , Freedman, Melvin , Kannourakis, George , Kinsey, Sally , Davis, Robert , Scarlata, Debra , Schwinzer, Beate , Zeidler, Cornelia , Welte, Karl
- Date: 2003
- Type: Text , Journal article
- Relation: American Journal of Hematology Vol. 72, no. 2 (Feb 2003), p. 82-93
- Full Text: false
- Reviewed:
- Description: Severe chronic neutropenia (SCN) is defined as an absolute neutrophil (ANC) of less than 0.5 x 10(9)/L, lasting for months or years. Congenital, cyclic, and idiopathic neutropenia are principal categories of SCN. Since 1994, the Severe Chronic Neutropenia International Registry (SCNIR) has collected data to monitor the clinical course, treatments, and disease outcomes for SCN patients. This report summarizes data for 853 patients, almost all treated with daily or alternate-day recombinant human granulocyte colony-stimulating factor (G-CSF or Filgrastim). G-CSF treatment increased the ANC overall from 0.34 x 10(9)/L +/- 0.018 pre-treatment to 3.70 x 10(9)/L +/- 0.18 during the first year of treatment. For most patients, the responses were durable with patients remaining on the same dose of G-CSF for many years. Long-term hematological observations showed stable mean leukocyte and neutrophil counts and gradually increasing hemoglobin levels. Thrombocytopenia developed in 4% of patients. As of January 1, 2000, myelodysplasia (MDS) or. acute myelogenous leukemia (AML) has occurred in 35 of 387 patients with congenital neutropenia with a cumulative risk of 13% after 8 years of G-CSF treatment. This event occurred without a predictable relationship to the duration or dose of G-CSF treatment. No patients with cyclic or idiopathic neutropenia developed MDS or AML. Other important adverse events included hepatomegaly, osteoporosis, vasculitis, glomerulonephritis, and deaths in 4 of 14 cases requiring splenectomy. Growth and development and the outcome of pregnancy appeared to be unaffected by G-CSF treatment. These data indicate that congenital, cyclic, and idiopathic neutropenia can be effectively treated with long-term GCSF. The risk of leukemia, osteoporosis, other potentially adverse events, and pregnancy outcome need to be further evaluated with continuing long-term observations. (C) 2003 Wiley-Liss, Inc.
- Description: C1