CNS Leukemia Treatment Clinical Practice Guidelines (2018)

International Lymphoma Radiation Oncology Group

Reviewed and summarized by Medscape editors

September 27, 2018

Guidelines on the treatment of CNS leukemia were released on September 1, 2018, by the ILROG.[1]

Lumbar puncture is the standard of care for evaluating CNS involvement in patients with newly diagnosed acute lymphoid leukemia (ALL), but in adults with newly diagnosed acute myeloid leukemia (AML), lumbar puncture is not recommended if there are no neurologic symptoms.

In patients who have AML and have risk factors for involvement of the CNS, lumbar puncture can be performed when systemic therapy is completed, to document remission.

In patients suspected of having leukemic involvement of the CNS, lumbar puncture with cytologic and flow cytometry analyses should be performed along with MRI of the craniospinal axis.

Effective CNS prophylaxis for ALL requires systemic and intrathecal-directed therapy. Radiation therapy (RT) should be rarely considered, on a case-by-case basis in patients who have high-risk features.

Methotrexate and cytarabine are the 2 primary chemotherapy agents for the prophylaxis and treatment of CNS leukemia.

RT should be considered for patients who have overt CNS leukemia at diagnosis and for patients who develop CNS leukemia at the onset of disease relapse, particularly when other CNS-directed therapies have failed.

For patients who are undergoing allogeneic hematopoietic stem cell transplantation, comprehensive RT to the CNS should be considered in those patients who have ALL or AML and have a history of CNS involvement.

A minimum of 2 weeks is recommended between the last intravenous or intrathecal administration of methotrexate or cytarabine and the initiation of CNS-directed RT. However, if RT is necessary because of symptoms, a shorter interval of 48 to 72 hours may be considered.

The choice of comprehensive RT (craniospinal irradiation) or limited RT to the CNS should depend on the expected long-term outcome for each patient.

A high suspicion of therapy-related neurotoxicity should be maintained for heavily pretreated patients who present with CNS-related symptoms.

The recommended RT dose can vary from 18 to 24 Gy. For patients with gross CNS disease, a dose of 23.4 Gy in 1.8-Gy fractions is recommended. In pediatric cases, a reduced dose of 18 Gy to the spine can be considered.


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