Treatment of Primary Lymphoma of Bone with CNS Involvement, Stage IV, in Patients Under 18
Clinical Scenario
This first-line protocol addresses pediatric patients (under 18 years) with mature aggressive B-cell lymphoma at stage IV and central nervous system involvement but no blasts detectable in the cerebrospinal fluid, or Burkitt leukemia that is CNS-negative.
Central nervous system involvement is the defining clinical feature of this high-risk subgroup. Stage IV disease with CNS involvement — even when cerebrospinal fluid blast counts are negative — requires a specifically structured, intensive treatment approach distinct from lower-stage presentations.
Treatment Approach (Partial Overview)
Two established intensive multi-course chemotherapy concepts are applied in this setting. Both incorporate sequential treatment phases that combine systemic chemotherapy with intrathecal therapy administration. The complete regimen sequence, agent selection, scheduling, and course structure are detailed in the full protocol.
Treatment Goals
Response is assessed at defined checkpoints: the protocol targets absence of blasts in the cerebrospinal fluid by the third treatment course, and absence of blasts in the bone marrow prior to the third course.
References
- Patients with CNS-involvement (stage IV mature B-NHL): P(IT) - AAZ124 - BBZ124 - CC - AAZ224 - BBZ224 - CC
- C1 Incomplete stage IV CNS positive and CSF negative B-AL CNS negative
- <18 years old at diagnosis.
- C1: COP - R-COPADM - R-COPADM2 - R-CYVE - R-CYVE - m1 - m2
- Non-response in the CNS is diagnosed in cases of persistence of CSF blasts during the third course.
- Bone marrow non-response is defined as persistent blasts prior to the third course.
View source ↗