Prognosis

In the US, the estimated 5-year relative survival rate for ALL is 72.6% (2015-2021 data).[10]

Outcome in adult patients is age-dependent. The estimated 5-year relative survival rate (2015-2021 data) is:[11]

  • 81.9% for patients ages <50 years

  • 45.3% for patients ages 50-64 years

  • 25.6% for patients ages 65 years and above

Prognostic factors

Negative prognostic features include older age, elevated white blood cell (WBC) count at presentation, failure to achieve a complete remission (CR), and adverse cytogenetic and molecular abnormalities.

Younger patients with WBC less than 30,000/microliter and who respond to treatment within 4 weeks have the best prognosis.[1][100][181][182][183][184]

Individual risk depends on a variety of clinical and biologic factors including:

  • Age: among children, those ages <1 year are considered very high risk, those ages ≥10 years are considered high risk, and those ages 1-9 years are considered standard risk.[86][185]​​ Adults ages >35 years are considered high risk, although the impact of age is a continuous variable.[87][88]

  • WBC count at presentation: a continuous variable, >30,000/microliter for B-ALL and >100,000/microliter for T-ALL are considered high risk for adults, and >50,000/microliter for B-ALL is considered high risk for children.[86]

  • Cytogenetic and molecular abnormalities. See Diagnostic criteria.​​

  • Immunophenotypic subtype: CD20 expression and early T-cell precursor (ETP)-ALL subtype (negative for CD5, CD8, and CD1a and positive for one or more myeloid/stem cell markers) is associated with a poor prognosis.[89][91]

  • Response to induction therapy: patients receiving induction therapy who do not achieve a CR, or who respond poorly, are considered high risk.

  • Presence of measurable residual disease (MRD; e.g., ≥0.01%) following induction therapy: persistent MRD after induction is associated with poor outcomes.[90]

Central nervous system (CNS) involvement

CNS involvement is a major complication of ALL occurring in 5% to 7% of patients at diagnosis; incidence is higher in patients with T-ALL (8%) and mature B-ALL (Burkitt lymphoma/leukemia, 13%).[9][55][56]​​[57][58]​ CNS involvement can lead to severe neurologic morbidity (i.e., cranial nerve palsies), and is a major obstacle to cure, accounting for up to 40% of initial relapses in clinical trials.[92][93][94]​ Studies have shown that use of intensive regimens in patients with CNS involvement at diagnosis results in similar outcomes (i.e., CR, disease-free survival, and overall survival) to patients without CNS involvement at diagnosis, particularly among adults.[55][56][95][96][97][98]​ However, outcomes are often poor in patients with CNS relapse (median overall survival <1 year).[99] Patients with CNS involvement (particularly CNS relapse) usually warrant consideration for postremission allogeneic SCT.[74][82]​​

Relapse/refractory disease

The prognosis in relapsed or refractory disease is generally poor. Median survival is less than 1 year, and less than 25% of patients survive in the long term.[147]

Long-term survival is dependent upon attaining a complete remission (CR) with subsequent allogeneic stem cell transplantation (SCT).

An individual's risk depends on a variety of clinical and biologic factors including:[186][187][188]

  • Age: younger patients are more likely to attain a CR and have better survival.

  • Long duration of first CR: survival is better for those whose duration of first remission is more than 2 years.

  • Site of relapse: if there is involvement of additional sites.

  • Status of disease at SCT: patients who underwent SCT in CR after salvage did better than those who were not in CR at the time of SCT. Patients who achieve MRD-negative remission with salvage therapy prior to SCT may also have better outcomes, but this has not been definitively established in a prospective trial.

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