Outcome and pathologic classification of children and adolescents with mediastinal large B-cell lymphoma treated with FAB/LMB96 mature B-NHL therapy

Gerrard, Mary, Waxman, Ian M., Sposto, Richard, Auperin, Anne, Perkins, Sherrie L., Goldman, Stanton, Harrison, Lauren, Pinkerton, Ross, McCarthy, Keith, Raphael, Martine, Patte, Catherine and Cairo, Mitchell S. (2013) Outcome and pathologic classification of children and adolescents with mediastinal large B-cell lymphoma treated with FAB/LMB96 mature B-NHL therapy. Blood, 121 2: 278-285. doi:10.1182/blood-2012-04-422709


Author Gerrard, Mary
Waxman, Ian M.
Sposto, Richard
Auperin, Anne
Perkins, Sherrie L.
Goldman, Stanton
Harrison, Lauren
Pinkerton, Ross
McCarthy, Keith
Raphael, Martine
Patte, Catherine
Cairo, Mitchell S.
Total Author Count Override 12
Title Outcome and pathologic classification of children and adolescents with mediastinal large B-cell lymphoma treated with FAB/LMB96 mature B-NHL therapy
Journal name Blood   Check publisher's open access policy
ISSN 0006-4971
1528-0020
Publication date 2013-01
Year available 2012
Sub-type Article (original research)
DOI 10.1182/blood-2012-04-422709
Volume 121
Issue 2
Start page 278
End page 285
Total pages 8
Place of publication Washington, DC, United States
Publisher American Society of Hematology
Collection year 2013
Language eng
Abstract Mediastinal large B-cell lymphoma (MLBL) represents 2% of mature B-cell non-Hodgkin lymphoma in patients ≤ 18 years of age. We analyzed data from childhood and adolescent patients with stage III MLBL (n = 42) and non-MLBL DLBCL (n = 69) treated with Group B therapy in the French-American-British/ Lymphome Malins de Burkitt (FAB/LMB) 96 study. MLBL patients had a male/female 26/16; median age, 15.7 years (range, 12.5-19.7); and LDH < 2 versus ≥ 2 x the upper limit of normal, 23:19. Six MLBL patients (14%) had < a 20% response to initial COP (cyclophosphamide, vincristine, and prednisone) therapy. Central pathology revealed approximately 50% with classical features of primary MLBL. Five-year event-free survival for the stage III MLBL and non-MLBL DLBCL groups was 66% (95% confidence interval [CI], 49%-78%) and 85% (95% CI, 71%-92%), respectively (P < .001; 14%). The 5-year overall survival in the 42 MLBL patients was 73% (95% CI, 56%-84%).We conclude that MLBL in adolescent patients is associated with significantly inferior event-free survival compared with stage III non-MLBL DLBCL and can be of multiple histologies. Alternate treatment strategies should be investigated in the future taking into account both adult MLBL approaches and more recent biologic findings in adult MLBL.
Keyword Mediastinal large B-cell lymphoma (MLBL)
B-cell non-Hodgkin lymphoma
Group B therapy
Treatment strategies
Q-Index Code C1
Q-Index Status Confirmed Code
Institutional Status UQ
Additional Notes Prepublished online: 13 November 2012.

Document type: Journal Article
Sub-type: Article (original research)
Collections: Official 2013 Collection
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