Primary Lymphoma of Bone in Children Under 18: What to Do When Standard-Risk ALL-Type BFM Treatment Does Not Achieve Response

This protocol addresses the specific situation of a pediatric patient — under 18 years old — with precursor B-cell lymphoblastic lymphoma (pB-LBL) presenting as primary lymphoma of bone, at stage I or II, who has undergone a standard-risk ALL-type BFM regimen and has not met the required early response benchmarks.

Patient population

Precursor B-cell lymphoblastic lymphoma (pB-LBL), including mixed lineage LBL (B/myeloid), with stage I or II disease; age younger than 18 years at diagnosis.

Previous line — confirmed non-response

The prior regimen was an ALL-type BFM protocol given as standard-risk treatment. Escalation to this protocol is indicated when the following response targets were not achieved by day 33: a reduction of at least 35% of the initial tumour volume; fewer than 5% blasts in the bone marrow; and no blasts in the cerebrospinal fluid.

Treatment approach (partial — full regimen behind the link)

Following confirmed non-response to the standard-risk regimen, the therapeutic strategy shifts to protocols designed for high-risk acute lymphoblastic leukaemia. Local irradiation may also form part of the overall plan.

The complete structured regimen, sequencing, and eligibility criteria are available via the link below.

Instant Access to Structured Evidence-Based Regimens

References

Standard risk group (SR): pB-LBL including mixed lineage LBL (B/myeloid) with stage I and II disease

<18 years old at diagnosis.

If a non-response is confirmed, these patients might be treated according to protocols for high risk ALL. Local irradiation may also be considered.

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