Second-Line Therapy in Extranodal Marginal Zone Lymphoma - Episode 1
Ariela Noy, MD:A 64-year-old woman with Sjögren’s syndrome develops masses near her right carotid. She has a workup that shows that she has bilateral carotid masses, some small lymph nodes, and a lesion in the left lung. Serial biopsies demonstrate that the carotid mass is marginal zone lymphoma and that there is a concordant B-cell infiltrate in the lung lesion.
At first, she is observed. However, the carotid lesion becomes more symptomatic. A decision is made to treat her with rituximab. After 4 weeks of therapy, she has resolution of her symptoms and serial imaging demonstrates that she has a near complete response. However, at 20 months, she develops a contralateral lung lesion, which also demonstrates the same B-cell infiltrate.
At 20 months, when she has progression of her disease, the physician decides to retreat her with rituximab. Unfortunately, she does not have a treatment response and a decision is made to start ibrutinib.
Marginal zone lymphoma is a relatively common type of indolent lymphoma, but it is not as common as follicular lymphoma. More physicians are comfortable with treating follicular lymphoma and sometimes miss the nuances between the 2 diseases.
Despite the fact that most patients with disseminated disease may feel anxious about their prognosis, their prognosis is actually quite excellent. We currently anticipate that patients like this will have a normal life expectancy. Some of them will either be treated initially or in follow up. Some will never be treated. Regardless, we expect them to live for decades.
Transcript edited for clarity.
A 64-Year-Old Woman With Advanced Extranodal MZL