This protocol addresses primary lymphoma of bone presenting as Burkitt leukemia (mature B-cell acute leukemia) in a patient under 18 years of age who has confirmed central nervous system (CNS) involvement with blasts detectable in the cerebrospinal fluid (CSF) at diagnosis.
The presence of CSF blasts at diagnosis defines a high-risk, CNS-positive subgroup. This distinguishes the case from CNS-negative disease and necessitates a specific protocol designed around this finding.
Management follows one of two established intensive multi-course concepts — one BFM-based, incorporating CNS-directed intrathecal chemotherapy from the initial phase, and an alternative that additionally includes a targeted antibody component — with full course sequencing and agent details available in the complete protocol below.
The key milestones for this protocol are clearance of blasts from the cerebrospinal fluid by the third course and clearance of blasts from the bone marrow before the third course. Persistence of CSF blasts into the third course, or persistence of bone marrow blasts prior to that point, defines non-response.