Treatment of Primary Lymphoma of Bone in Patients Under 18 with T-Cell or Precursor B-Cell Lymphoblastic Lymphoma
Clinical Scenario
This protocol applies to paediatric patients Age < 18 years who present with primary lymphoma of bone in the context of lymphoblastic lymphoma:
- T-cell lymphoblastic lymphoma (T-LBL) — any stage, including mixed lineage (T/myeloid or T/B)
- Precursor B-cell lymphoblastic lymphoma (pB-LBL) — stage III or IV disease, including mixed lineage (B/myeloid)
- Also covers cases where CNS or bone marrow status is unknown at diagnosis
Treatment Approach (Overview)
Management is based on an ALL-type BFM regimen incorporating a re-intensification phase, structured across induction, consolidation, re-induction, and oral maintenance. The specific phases, agents involved, and the complete eligibility and sequencing criteria are detailed in the full protocol.
Response Targets — Day 33
Reduction of at least 35% of the initial tumour volume
Fewer than 5% blasts remaining in the bone marrow
No blasts detectable in the cerebrospinal fluid
References
- High Risk Group (HR): All stages of T-LBL including mixed lineage LBL (T/myeloid or T/B)
- pB-LBL including mixed lineage LBL (B/myeloid) with stage III and IV disease
- all LBL with an unknown CNS and/or BM status
- <18 years old at diagnosis.
- Patients with precursor lymphoid neoplasms are treated according to the ALL-type treatment regimen (see Appendix A).
- All high-risk patients receive a re-intensification phase (protocol II).
- 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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