Diagnosis of Indolent Lymphomas: MZL vs FL

Anthony Mato, MD, MSCE: Let’s delve into patient presentations. I would argue that patients with follicular lymphoma most frequently present with an asymptomatic lymph node that was palpated, or an imaging study that was performed for another reason where some abnormal lymphadenopathy was observed. Very rarely do I see a patient with a follicular lymphoma who presents with severely symptomatic disease. In general, these indolent lymphomas are quiet diseases that often don’t require treatment in the beginning. The marginal zone lymphomas are somewhat different; their presentations are so variable. It sometimes seems funny lumping them all under the same diagnostic category. You mentioned splenic marginal zone lymphoma. Could you comment on how that typically presents in your practice?

Lori A. Leslie, MD: I agree, marginal zone lymphoma is very challenging. The fact that we’ve historically lumped all these indolent lymphomas together in clinical trials is changing pretty rapidly in the era of targeted medicine. Not only do they present differently, but they have different molecular drivers. With splenic marginal zone lymphoma, I usually see asymptomatic lymphocytosis, or an enlarged spleen that may or may not be symptomatic, other cytopenias, maybe from splenic sequestration or extensive bone marrow involvement. I typically can check peripheral blood and pick up a clone that way. Bone marrow biopsy typically confirms the diagnosis. Bone marrow involvement is very common. Sometimes there’s nothing in the bone marrow and just an enlarged spleen, and if it’s symptomatic, I’ll send a patient for a splenectomy. That’s not a common patient, but in some situations, I don’t have even the definitive diagnosis until we have the diagnostic and therapeutic splenectomy.

Anthony Mato, MD, MSCE: What about the extranodal cases? We’ve talked about the eye, the gastric tract, lungs, and skin. The location of the disease speaks volumes about how these patients are presenting. They’re so variable in their presentation. Do you have any insights into the clinical presentation of extranodal marginal zone lymphomas?

Lori A. Leslie, MD: Yes. I find the cutaneous-only type tends to be very indolent. I have a handful of patients who just spontaneously regress. Other patients will have symptomatic disease, but only 1 location, and just need some radiation. Then maybe 20 years later, they will have another lesion pop up. Then there are other patients who have multifocal disease, and those patients may benefit from rituximab, for example, in rare situations. That tends to be the most indolent. With gastric, I find that the pulmonary extranodal marginal zone lymphoma is a little more challenging in general because patients usually present with more symptomatic disease from the lung involvement. So I usually end up treating those patients more like a systemic nodal marginal zone lymphoma. But overall, the infectious work-up, as we mentioned, and then typically the disease organ-targeted therapy, commonly with radiation, with or without a CD20 monoclonal antibody, can give patients potential for cure.

Anthony Mato, MD, MSCE: We have talked over and over about how these patients are presenting in an indolent fashion. I would say many patients are seen, observed, and not treated from the time of their diagnosis. Would you agree with that?

Lori A. Leslie, MD: I would. In our practices, we’re probably a little more enriched for patients who have more aggressive diseases, just from being referral centers. With that being said, the majority of patients I initially meet are appropriate for watch and wait.

Transcript Edited for Clarity