For pediatric patients younger than 18 diagnosed with primary lymphoma of bone presenting as precursor B-cell lymphoblastic lymphoma (pB-LBL) — including the mixed lineage LBL (B/myeloid) subtype — with stage I or II disease, a structured first-line approach is defined for this standard-risk group.

Clinical Scenario

Standard risk group: precursor B-cell lymphoblastic lymphoma (including mixed lineage LBL B/myeloid) with stage I or II disease, in patients younger than 18 years at diagnosis.

Treatment Approach

Patients in this group are treated according to an ALL-type regimen following a BFM protocol framework, structured across distinct induction, consolidation, and oral maintenance phases. The complete agent sequence and full protocol details are available via the link below.

Response Goals (Day 33)

Treatment targets a reduction of at least 35% of the initial tumor volume by day 33, with fewer than 5% blasts in the bone marrow and no blasts in the cerebrospinal fluid at that assessment point.

References

Standard risk group (SR): pB-LBL including mixed lineage LBL (B/myeloid) with stage I and II disease
<18 years old at diagnosis.
Patients with precursor lymphoid neoplasms are treated according to the ALL-type treatment regimen (see Appendix A).
Patients receive maintenance therapy for a total of 24 months therapy duration calculated from the first day of the cytoreductive prephase.
Reduction of at least 35% of the initial volume
Non-responders are patients with less than 35% volume response/regression at day 33, and/or persistence of >5% blasts in the bone marrow and/or persistence of blasts in the CSF at day 33.

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Treatment of Primary Lymphoma of Bone in Patients Under 18 with Stage I or II Precursor B-Cell Lymphoblastic Lymphoma

Clinical Scenario

Treatment Approach

Response Goals (Day 33)

References