Canterbury DHB
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Patients who are given supportive care have a median survival of only 2-3 months.
Most trials of intensive treatment in Philadelphia-negative adult ALL have produced a complete remission rate of 70-80% and a 5 year event free survival of 30-40%. Adolescents and young adults (<30) do better than older patients. There is evidence that superior results are achieved if such patients are given paediatric protocols. For example, in trials conducted between 1988 and 1995, young adults aged 16-21 entered into COG trials (a children's oncology trials group) did better than patients entered into CALGB trials (an adult group). CR rates were the same but EFS at 6 years was 64% for COG and 38% for CALGB. The reasons for these differences are not clear. With current treatment schedules there is no difference in outcome between precursor T cell and precursor B cell ALL. See Lafage-Pochitaloff, M., et al. (2017). "Impact of cytogenetic abnormalities in adults with Ph-negative B-cell precursor acute lymphoblastic leukemia." Blood 130(16): 1832.
Philadelphia-positive adult ALL historically had a very poor prognosis, but this has been dramatically improved by using tyrosine kinase inhibitors – first imatinib, but now more usually dasatinib. Most children and adults who are fit enough will receive myeloablative allogeneic SCT in first remission. See Igwe, I. J., et al. (2017). "The presence of Philadelphia chromosome does not confer poor prognosis in adult pre-B acute lymphoblastic leukaemia in the tyrosine kinase inhibitor era – a surveillance, epidemiology, and end results database analysis." British Journal of Haematology 179(4): 618-626.
Patients with mature B-cell ALL Burkitt-like leukaemia have a good prognosis provided they can tolerate the high intensity treatment schedules required. EFS at 5 years is up to 80%.
In adult patients with precursor B-cell or precursor T-cell ALL, certain features confer a poor prognosis.
Table 2 – Adverse risk factors in precursor T-cell or B-cell ALL |
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Adult patients with ALL who lack any of the above features are in a minority but do quite well with intensive chemotherapy with an event free survival (EFS) of 60% at 5 years. By contrast patients with any of the above features have an EFS at 5 years of between 18-28%.
It has become clearer that the best leukaemia free survival results for adolescents and young adults are obtained by treatment on paediatric ALL protocols. However, some of these are nearing maximal toxicity and their safety in older patients needs to be carefully evaluated in formal trials.
Older adults who are deemed unsuitable for the high intensity treatment designed for adolescents and younger adults would usually be offered intensive treatment of the kind popularised in UK ALL trials such as UKALL14. Less fit older adults (eg > 60years) may be offered intermediate intensity schedules such as modified OPAL or more palliative treatments. The most appropriate treatment is decided by the Consultant in conjunction with that patient. Factors that need to be considered include patient’s age, performance status, co-morbidities, and the patient’s wishes. Elderly patients with Philadelphia positive ALL may achieve a remission with steroids and imatinib alone.
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Topic Code: 4407
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