title: Burkitt leukaemia/lymphoma: R-CODOX-M/R-IVAC remains gold standard treatment in BL.
abstract: BACKGROUND
  Sporadic Burkitt lymphoma (BL), characterised by translocation-associated C-MYC upregulation is a rare, aggressive lymphoma with a cure rate up to 90 % using the R-CODOX-M/R-IVAC (RCRI) protocol. RCRI is active in HIV-associated BL in combination with HAART. The WHO classification system defines lymphomas intermediate between DLBCL and BL, in which lymphomas with t(14;18)(q32;q21) and C-MYC-carrying translocation, i.e. 'double-hit' are included (BL-DH), and these patients are conventionally treated with RCRI.
  
  
  RESULTS
  We describe the SJH experience of 25 patients with BL, BL + HIV and BL-DH treated with RCRI between 2002 and 2011. Twelve BL patients (8M/4F), median age 49.1 years (range 20-73 years); of whom 9 had extensive disease, including 8 with marrow and 2 with CNS involvement. Eleven patients remain in remission at 80.5 months (range 37-147 months) from completion of treatment and one died of progressive BL giving an OS of 91.6 % at 1 year with no late relapses. Eight patients with BL + HIV were treated (6M/2F) with a median age 40.25 years (range 24-64). Five remain in complete remission (CR) at 65 months (range 13-109 months), three patients died, two of progressive disease and one of treatment-associated hepatotoxicity in CR. Five patients with BL-DH were included; (3M/2F), age 47.8 years (range 42-55 years); and all patients died of progressive disease, 4 on RCRI therapy and a further patient despite an allogeneic transplantation.
  
  
  CONCLUSIONS
  These results confirm that RCRI is an effective treatment in adults with BL and BL + HIV and remains the gold standard against which other regimens should be compared. We confirm the poor prognosis found in BL-DH, indicating new treatment approaches are needed for this sub-group which should be identified at diagnosis by FISH analysis.
authors.affiliation: Department of Haematology, St James's Hospital, Dublin 8, Ireland. lismyth@tcd.ie.
authors.forename: L
authors.initials: L
authors.lastname: Smyth
authors.affiliation: Department of Haematology, St James's Hospital, Dublin 8, Ireland.
authors.forename: P V
authors.initials: PV
authors.lastname: Browne
authors.affiliation: Department of Haematology, St James's Hospital, Dublin 8, Ireland.
authors.forename: E
authors.initials: E
authors.lastname: Conneally
authors.affiliation: Department of Haematology, St James's Hospital, Dublin 8, Ireland.
authors.forename: C
authors.initials: C
authors.lastname: Flynn
authors.affiliation: Department of Haematology, St James's Hospital, Dublin 8, Ireland.
authors.forename: P
authors.initials: P
authors.lastname: Hayden
authors.affiliation: Department of Histopathology, AMNCH, Dublin 24, Ireland.
authors.forename: M
authors.initials: M
authors.lastname: Jeffers
authors.affiliation: Department of Haematology, St James's Hospital, Dublin 8, Ireland.
authors.forename: D
authors.initials: D
authors.lastname: O'Brien
authors.affiliation: Department of Cancer Molecular Diagnostics, St James's Hospital, Dublin 8, Ireland.
authors.forename: F
authors.initials: F
authors.lastname: Quinn
authors.affiliation: National Centre for Medical Genetics, Our Lady's Children's Hospital, Crumlin, Dublin 12, Ireland.
authors.forename: J
authors.initials: J
authors.lastname: Kelly
authors.affiliation: Department of Haematology, Midlands Regional Hospital, Tullamore, Co. Offaly, Ireland.
authors.forename: M
authors.initials: M
authors.lastname: Perera
authors.affiliation: Department of Haematology, Midlands Regional Hospital, Tullamore, Co. Offaly, Ireland.
authors.forename: G M
authors.initials: GM
authors.lastname: Crotty
authors.affiliation: Department of Haematology, University Hospital Limerick, Limerick, Ireland.
authors.forename: M
authors.initials: M
authors.lastname: Leahy
authors.affiliation: Department of Haematology, University Hospital Waterford, Waterford, Ireland.
authors.forename: B
authors.initials: B
authors.lastname: Hennessy
authors.affiliation: Department of Haematology, University Hospital Waterford, Waterford, Ireland.
authors.forename: F
authors.initials: F
authors.lastname: Jackson
authors.affiliation: Department of Haematology, University Hospital Waterford, Waterford, Ireland.
authors.forename: M
authors.initials: M
authors.lastname: Ryan
authors.affiliation: Department of Haematology, St James's Hospital, Dublin 8, Ireland.|Department of Cancer Molecular Diagnostics, St James's Hospital, Dublin 8, Ireland.
authors.forename: E
authors.initials: E
authors.lastname: Vandenberghe
mesh_terms: D000328:Adult; D000368:Aged; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D023241:Antiretroviral Therapy, Highly Active / Q000379:methods; D002051:Burkitt Lymphoma / Q000188:drug therapy*; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D016896:Treatment Outcome; D055815:Young Adult
keywords: Burkitt leukaemia/lymphoma; Classic Burkitt lymphoma; Double hit; HIV-associated lymphoma
references.citation: J Clin Oncol. 2007 Feb 10;25(5):579-86
references.pmid: 17242396
references.citation: Ann Oncol. 2002 Aug;13(8):1264-74
references.pmid: 12181251
references.citation: Blood. 2010 Apr 15;115(15):3017-24
references.pmid: 20130244
references.citation: J Infect Dis. 2009 Jul 1;200(1):79-87
references.pmid: 19476437
references.citation: Blood. 2004 Nov 15;104(10):3009-20
references.pmid: 15265787
references.citation: Leuk Lymphoma. 2012 Oct;53(10):1966-70
references.pmid: 22938139
references.citation: Blood. 2009 Sep 10;114(11):2273-9
references.pmid: 19597184
references.citation: J Clin Oncol. 1996 Mar;14(3):925-34
references.pmid: 8622041
references.citation: Hematology Am Soc Hematol Educ Program. 2008;:341-8
references.pmid: 19074108
references.citation: N Engl J Med. 2013 Nov 14;369(20):1915-25
references.pmid: 24224624
references.citation: Am J Clin Pathol. 2010 Aug;134(2):258-65
references.pmid: 20660329
references.citation: Cancer Cytopathol. 2011 Aug 25;119(4):263-71
references.pmid: 21560252
references.citation: Am J Clin Pathol. 2010 Jul;134(1):127-38
references.pmid: 20551277
references.citation: Curr Hematol Malig Rep. 2011 Mar;6(1):58-66
references.pmid: 21191675
references.citation: Br J Haematol. 2011 Jan;152(2):175-81
references.pmid: 21092025
references.citation: J Clin Pathol. 2011 Sep;64(9):802-8
references.pmid: 21593346
references.citation: Br J Surg. 1958 Nov;46(197):218-23
references.pmid: 13628987