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CNS involvement in ALL

Last reviewed dd mmm yyyy. Last edited dd mmm yyyy

Authoring team

CNS involvement at the time of diagnosis is observed in <5% of children and in <10% of adults with ALL (1).

CNS prophylaxis has been identified as the single best approach in treating patients with CNS leukaemia.

Without CNS prophylaxis

  • 50-75% of patients eventually will develop CNS disease
  • CNS recurrence occurs in approximately 30 - 50% of patients who have achieved complete remission (1)

CNS recurrence is associated with a poor prognosis (1).

CNS relapse has been observed in 30-40% of initial relapses in some studies (1).

The following factors are associated with an increased risk of CNS relapse:

  • a T-cell immunophenotype
  • hyperleucocytosis
  • high-risk genetic abnormalities
  • presence of leukaemic cells in cerebrospinal fluid

Due to the associated acute and late complications (e.g. - second cancers, late neurocognitive deficits, and endocrinopathy), cranial irradiation is limited to patients who are at high risk of a CNS relapse (1,2).

Intrathecal and systemic chemotherapy have replaced cranial irradiation (1).

  • triple intrathecal therapy with methotrexate, cytarabine, and hydrocortisone is effective in preventing CNS relapse (2)

Reference:

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