Pathogenetic, Clinical, and Prognostic Features of Adult t(4;11)(q21;q23)/MLL-AF4 Positive B-Cell Acute Lymphoblastic Leukemia

Table 4

Clinical outcome of t(4;11)(q21;q23)/MLL-AF4 positive ALL in different cooperative trials worldwide.

Study	Year	No. Patients; (Age)	% of patients t(4;11)/MLL-AF4 positive	Treatment strategy	% of patients undergoing HSCT intensification	Global outcome; (OS and DFS)	t(4;11)/MLL-AF4 positive ALL outcome
MRC UKALLXII/ECOG E2993 [30]	2007	1522 (15–65)	5.4%	Intensification with HSCT for Ph+ and for patients younger than 50 years with HLA-matched family donor	21%	5y-DFS: 38%(^a); 5y-OS: 43%(^a)	5y-DFS: 24%; 5y-OS: 24%
GIMEMA LAL0496 [32]	2003	403 (15–60)	6%	Intensification with HSCT only for Ph+ patients	20%	5y-DFS: 31%; 5y-OS: 31%	5y-CCR: 15%; 5y-OS: 23%
GMALL 04/87–89 [33]	1998	611 (15–65)	3.6%	Intensification with HSCT for younger high risk patients with HLA-matched family donor	na	5y-CCR: 45%; 5y-OS: 40%	5y-CCR: 40%; 5y-OS: 41%
NILG-ALL 09/00 [35]	2009	280(16–65)	7.3%	Intensification with HSCT in patients MRD+ after consolidation	31%	5y-OS: 34%	5y-OS 27%(^b)
LALA94 [38]	2006	922 (15–55)	6%	Intensification with HSCT in high risk and CNS+ patients	19%	5y-DFS: 30%; 5y-OS: 33%	5y-DFS: 30%; 5y-OS: 38%
GRAALL-2003 [34]	2009	225 (15–60)	9.5%	All patients were Ph negative. Intensification with HSCT in high risk patients	31%	3.5y-DFS: 55%; 3.5y-OS: 60%	3.5y-DFS: 52%(^b); —
PETHEMA ALL-93 [39]	2005	222 (15–50)	4%	Intensification with HSCT for patients with HLA-matched family donor	31%	5y-DFS: 35%; 5y-OS: 34%	Same results

(^a)data relative at Ph negative patients; (^b)data relative at clinical outcome of all non-Ph+ high-risk patients evaluated, including t(4;11)/MLL-AF4 positive patients. OS: overall survival; DFS: disease-free survival; CCR: survival in continuous complete remission; CR: complete remission; HSCT: allogeneic hematopoietic stem cell transplantation; Ph+: Philadelphia-positive patients; MRD: minimal residual disease; CNS: central nervous system; MRC: British Medical Research Council; ECOG: Eastern Cooperative Oncology Group; GIMEMA: Gruppo Italiano Malattie EMatologiche dell’Adulto; GMALL: German Multicenter study group for treatment of adult Acute Lymphoblastic Leukemia; NILG: Northern Italy Leukemia Group; LALA: France-Belgium Group for Lymphoblastic Acute Leukemia in Adults; GRAALL: Group for Research on Adult Acute Lymphoblastic Leukemia (including the former France-Belgium Group for Lymphoblastic Acute Leukemia in Adults, the French Western-Eastern Group for Lymphoblastic Acute Leukemia, and the Swiss Group for Clinical Cancer Research); PETHEMA: Programa para el Estudio del la Terapeutica en Hemopatía Maligna; na: not available.