NCCN Updates Guidelines for Acute Lymphoblastic Leukemia

03/15/18

The National Comprehensive Cancer Network (NCCN) has published updates to their guidelines for acute lymphoblastic leukemia (ALL), which include new treatment options for Philadelphia (Ph)-positive, Ph-negative, and relapsed or refractory disease.

In the diagnosis section of the guideline, a footnote was modified to make reference of testing for gene abnormalities at diagnosis to improve risk stratification. “The Ph-like phenotype is associated with recurrent gene fusions and mutations that activate tyrosine kinase pathways and includes gene fusions involving ABL1, ABL2, CRLF2, CSF1R, EPOR, JAK2, or PDGFRB and mutations involving FLT3, IL7R, SH2B3, JAK1, JAK3, and JAK2 (in combination with CRLF2 gene fusions),” footnote G reads.

For adults with Ph-positive disease, tyrosine kinase inhibitor (TKI) therapy plus corticosteroids was added as a treatment option. For Ph-negative disease in adolescents and young adults in need of consolidative therapy, footnote AA reads “The prognostic significance of minimal residual disease (MRD) positivity may be regimen, ALL subtype, and/or ALL risk dependent. MRD time points and levels prompting allogeneic hematopoietic cell transplant (HCT) should be guided by the specific treatment protocol being used. In general, MRD positivity at the end of induction predicts high relapse rates and should prompt evaluation for allogeneic HCT. Therapy aimed at eliminating MRD prior to allogeneic HCT is preferred when possible.”

For patients with relapsed or refractory ALL, HCT should be considered after all treatment options for Ph-positive and Ph-negative disease. The blinatumomab indication for Ph-positive disease was modified; after failure of two TKIs, patients should be identified as TKI intolerant or refractory.

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Fludarabine-based regimens were added for patients with relapsed or refractory Ph-negative ALL. Among the added regimens were FLAG-IDA (fludarabine, cytarabine, granulocyte colony-stimulating and idarubicin) and FLAM (fludarabine, cytarabine, and mitoxantrone).

In the section for older adults (aged at least 65 years) with ALL, a couple of induction regimens were added for Ph-positive disease: nilotinib plus steroids as well as GRALL (doxorubicin, vincristine, dexamethasone, cytarabine, and cyclophosphamide).—Zachary Bessette