Canterbury DHB

Context

Prognosis, Risk Factors and the Decision to Treat

In This Section

Prognosis

Risk Factors

The Decision to Treat

Prognosis

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%.

Risk Factors

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

  • Age > 35 years
  • WBC at diagnosis more than 30 x109/L
  • Presence of the Philadelphia chromosome or BCR-ABL
  • t (4;11) translocation
  • Failure to enter CR by Day +28

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%.

The Decision to Treat

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.

About this Canterbury DHB document (4407):

Document Owner:

Peter Ganly (see Who's Who)

Issue Date:

November 2018

Next Review:

November 2021

Keywords:

Note: Only the electronic version is controlled. Once printed, this is no longer a controlled document. Disclaimer

Topic Code: 4407