Treatment of Chronic Lymphocytic Leukemia with Central Nervous System Involvement
Central nervous system (CNS) involvement in chronic lymphocytic leukaemia (CLL) is an uncommon but clinically significant presentation that requires careful evaluation and a tailored approach.
Clinical Scenario
CLL presentation in the CNS is rare. When it occurs, Richter transformation must first be excluded, as CNS disease can arise in that context.
This protocol addresses CLL with confirmed central nervous system involvement, where systemic therapy must account for CNS penetration.
Treatment Approach
Targeted therapy forms the basis of treatment in this setting, with agents selected in part for their ability to cross the blood–brain barrier.
The full regimen — including agent selection, sequencing, and duration guidance — is outlined in the structured protocol. No consensus currently exists on optimal treatment duration in this scenario.
References
- CLL presentation in the central nervous system (CNS) is rare.
- When it occurs, it is not infrequently in the context of Richter syndrome, which should be excluded.
- Patients with CNS presentation can be treated with targeted therapy, including BTKi or BCL2i, but no consensus exists on optimal duration (GRADE 2D).
- The first-generation BTKi, ibrutinib, has been shown to cross the blood–brain barrier and has been used successfully in CNS-involved CLL.
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