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?
Would your approach differ for a 30 year old and a 70 year old?
Answer from: Medical Oncologist at Academic Institution
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 b...
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 contin...
Answer from: Medical Oncologist at Academic Institution
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...