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Acute Lymphoblastic Leukemia

 

 

 

Risk Stratification

 

To determine the correct therapy, it is important to determine the patient’s risk of future relapse and ultimately death from leukemia.

Most children with ALL will go into remission, stay in remission, never relapse and become long-term survivors and with appropriate therapy.

The challenge is to differentiate “bad acting” leukemia as separate from the majority of patients who will have “good acting” leukemia so therapy can be tailored appropriately.

Two features continue to show prognostic significance at the time of diagnosis:

  • Age
  • Initial WBC

 

Favorable Prognosis

(traditionally “Standard Risk”)
  • Age 1-10 years

 

  • WBC <50 x 109/L

 

  • CNS Negative

 

Unfavorable Prognosis
  • Age <1 year (Infant ALL) – 20-30% long term survival

 

  • Age >10 years

 

  • WBC > 50 x 109/L

 

 

With contemporary clinical research protocols by large cooperative groups such as the Children’s Oncology Group, new prognostic factors have been found that better reflect the underlying biology.

 

Examples of Unfavorable features of ALL:

Standard-Risk “High” ALL
  • Any patient with a slow-early response to chemotherapy.

 

  • MLL gene translocation with a rapid early response to chemotherapy (in remission by day 15 of initial chemotherapy)

 

  • CNS3

 

  • Pretreated with corticosteroids before investigations complete.

High-Risk ALL
  • T-ALL

 

  • Any Patient Over 10 years of Age

 

  • Any Patient Between 1-10 years of Age with WBC >50

 

  • Testicular Disease

 

Very High-Risk ALL
  • Infant ALL

 

  • Presence of Philadelphia Chromosome

 

  • Failure to achieve remission in first month of chemotherapy (Induction Failure)

 

  • Hypodiploidy (<44 chromosomes)

 

  • MLL gene translocation with slow early response to chemotherapy

 

Increasingly use of either flow cytometry or molecular techniques to determine minute amounts of leukemia cells present during treatment (called minimal residual disease) will further define patients at higher risk of relapse and in need of intensification or different therapies.

 

 

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