How would you approach therapy for a patient with symptomatic secondary CNS marginal zone lymphoma, but with low-volume systemic disease that would otherwise not warrant therapy?

CNS marginal zone lymphoma, or in the case of CNS involvement by LPL/WM known as Bing-Neel syndrome, is uncommon and clinically heterogeneous. Treatment approaches depend on rigorously excluding aggressive lymphoma (treated differently), the pattern of involvement (parenchymal or leptomeningeal or both), disease biology (MYD88, p53, eg), as well as patients’ clinical characteristics.

For symptomatic parenchymal disease in young fit patients, a course of HDMTX-based chemotherapy is reasonable, re...

Has this person had a careful retinal exam? I had a case like this once where the CSF disease was low/asymptomatic, but had visual changes due to retinal disease. In that case, I gave ibrutinib 560 mg, since I knew it would get into the CSF. Once the retina improved, we had a discussion about continuing vs stopping - opted to stop due to bruising. This is a long way of saying I probably would not treat as would periodically do LP, MRI, and eye exam. If treating, would give ibrutinib vs R2 - mayb...

Secondary involvement of the CNS with marginal zone lymphoma is very rare. In my view, because it is symptomatic, it needs to be treated. No standard approach to treating CNS marginal zone lymphoma has been developed. A variety of treatments have been attempted with none emerging as clearly superior. It has recently been reported that primary CNS MALT lymphoma is responsive to chemotherapy (Desjardins et al., PMID 35725885); however, data on secondary CNS involvement are limited. A distinction i...