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.