What should I prescribe a young patient with a rare composite lymphoma diagnosis?

Professor of Medicine
Feinberg School of Medicine, Robert H. Lurie Comprehensive Cancer Center
Northwestern University
Division of Hematology/Oncology
Chicago, IL

This month, Jane Winter, MD, talks about how she would treat a young patient with a rare composite lymphoma diagnosis.

CLINICAL DILEMMA

I have an 18-year-old female patient who presented with syncope. A comprehensive workup showed a bulky right lung mass invading into the right atrium with an intra-atrial mass, as well as liver and kidney lesions. She also has severe vena cava syndrome due to the mass. A lung biopsy was performed; it showed “composite lymphoma with primary mediastinal lymphoma and focal involvement of classical Hodgkin lymphoma.” The pathology report was also reviewed by physicians at the National Institutes of Health who confirmed the diagnosis.

We started her on DA-EPOCH-R, and she finished the first cycle last week. I wanted to get your thoughts about the treatment of this rare entity and also any recommendations.

EXPERTS MAKE THE CALL

What a challenging case! Whereas primary mediastinal B-cell lymphoma (PMBCL) and Hodgkin lymphoma share many features and are biologically similar, I agree with your strategy of DA-EPOCH-R. It has been shown to be effective therapy in PMBCL as well as in grey zone lymphomas, part of the spectrum of disease to which your case belongs. Although there aren’t published data on this regimen in classical Hodgkin lymphoma, given its activity in grey zone lymphomas and PMBCL, I am hopeful it will be effective for your patient. CNS prophylaxis will be important because of the many extranodal sites and renal involvement. It’s always problematic to combine CNS prophylaxis with DA-EPOCH-R, and it generally comes down to IT methotrexate (MTX) with each cycle, although the risk of parenchymal brain disease persists. I’ve contemplated adding a few cycles of high-dose MTX at 3.5 gm/m2 at the completion of therapy or after achievement of a remission, but I haven’t yet pursued that strategy. There is the concern that integrating high-dose methotrexate with DA-EPOCH-R could compromise the “dose adjustments,” if it is scheduled on day 15 as we do when combining it with R-CHOP.

If you do achieve a CR based on PET/CT after three or four cycles, I’d strongly consider integrating some high-dose MTX at some point.

Expert’s Note: A group from the Department of Lymphoma/Myeloma at MD Anderson published a letter to the editor giving mid-cycle high-dose methotrexate in a small number of patients with secondary CNS involvement (Chihara D, Fowler NH, Oki Y, et al. Dose-adjusted EPOCH-R and mid-cycle high dose methotrexate for patients with systemic lymphoma and secondary CNS involvement. Br J Haematol. 2016 August 9. [Epub ahead of print]).

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