ABSTRACT
While initial therapy of mantle cell lymphoma (MCL) is not standardized, bendamustine-rituximab (BR) is commonly used in older patients. Rituximab (R) maintenance following induction is often utilized. Thus, the open-label, randomized phase II ECOG-ACRIN Cancer Research Group E1411 trial was designed to test two questions: 1) Does addition of bortezomib to BR induction (BVR) and/or 2) addition of lenalidomide to rituximab (LR) maintenance improve progression-free survival (PFS) in patients with treatment-naïve MCL? From 2012-2016, 373 previously untreated patients, 87% ≥ 60 years old, were enrolled in this trial. At a median follow up of 7.5 years, there is no difference in the median PFS of BR compared to BVR (5.5 yrs vs. 6.4 yrs, HR 0.90, 90% CI 0.70, 1.16). There were no unexpected additional toxicities with BVR treatment compared to BR, with no impact on total dose/duration of treatment received. Independent of the induction treatment, addition of lenalidomide to rituximab did not significantly improve PFS, with median PFS in R vs LR (5.9 yrs vs 7.2 yrs, HR 0.84 90% CI 0.62, 1.15). The majority of patients completed the planned 24 cycles of LR at the scheduled dose. In summary, adding bortezomib to BR induction does not prolong PFS in treatment-naïve MCL, and LR maintenance was not associated with longer PFS compared with rituximab alone following BR. Nonetheless, the > 5 year median PFS outcomes in this prospective cooperative group trial indicate the efficacy of BR followed by rituximab maintenance as highly effective initial therapy for older MCL patients. (NCT01415752).
ABSTRACT
BACKGROUND Angioedema is a vascular reaction of the soft tissues or mucosa, with localized increased permeability of blood vessels. Patients with late-onset angioedema without urticaria have an increased risk of non-Hodgkin lymphoma. We present a case of late-onset angioedema that demonstrates that it is sometimes necessary to treat an indolent malignancy to address the symptoms of a secondary condition. CASE REPORT A 68-year-old man presented to the Emergency Department with distressing swelling of his tongue and lips. No urticaria was observed and the remainder of the physical examination was unremarkable. The patient's past medical history included chronic thrombocytopenia for the last 1.5 years, which had been asymptomatic. Routine laboratory testing revealed pancytopenia. The patient was referred to the Oncology Department, where he was diagnosed with splenic marginal zone lymphoma. A careful review of the patient's past medical history revealed 3 episodes of soft tissue swelling of the lower limbs and 2 episodes of unexplained colicky abdominal pain. The patient was started on maintenance therapy of danazol, which prevented further episodes of angioedema. He later underwent splenectomy to improve his pancytopenia and to treat his lymphoma. In the postoperative period, the patient discontinued the danazol therapy. Three months after the splenectomy, he was asymptomatic and had not had any further angioedema episodes, and his laboratory values showed he was in remission. CONCLUSIONS In this case, late-onset angioedema with recurrent episodes of soft tissue swelling was associated with underlying hematologic malignancy. The patient's angioedema resolved when the malignancy was treated.