Primary Lymphoma of Bone in T-LBL or Stage III–IV pB-LBL (Age <18): What to Do When the ALL-Type BFM Regimen Does Not Achieve Response
Clinical Scenario
This protocol addresses primary lymphoma of bone in paediatric patients — those under 18 years at diagnosis — presenting with T-cell lymphoblastic lymphoma at any stage, or with precursor B-cell lymphoblastic lymphoma at stage III or IV disease.
Patient Population
Applicable to T-LBL at any stage (including mixed lineage T/myeloid or T/B), pB-LBL with stage III or IV disease (including mixed lineage B/myeloid), and all lymphoblastic lymphoma with unknown CNS or bone marrow status — all diagnosed at age under 18.
Prior Treatment & Failure Condition
Patients in this scenario have received an ALL-type BFM regimen with re-intensification as the previous line. This protocol is indicated when that treatment line fails to achieve a reduction of at least 35% of the initial tumor volume by day 33, or fails to reach targets of less than 5% blasts in the bone marrow and no blasts in the cerebrospinal fluid at day 33.
Treatment Approach (Partial Overview)
When non-response to the prior regimen is confirmed, management follows protocols designed for high-risk acute lymphoblastic leukemia. Local irradiation may also be considered.
The complete regimen, full eligibility criteria, and sequencing are detailed in the structured protocol below.
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.
- 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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