This phase II study evaluated time-limited (24 cycles) treatment with ibrutinib and ixazomib in newly diagnosed (NDWM; n = 9) and relapsed/refractory (RRWM; n = 12) Waldenström macroglobulinaemia (WM). The overall response rate (ORR) was 76.2% (n = 16) in 21 evaluable patients with no patient achieving a complete response (CR). The median duration of treatment was 15.6 months, and after a median follow-up time of 25.7 months, the median progression-free survival (PFS) was 22.9 months. While the primary end-point was not met (CR rate at any time) and 28.5% discontinued treatment due to toxicity, ibrutinib plus ixazomib led to a clinically meaningful ORR and PFS. Combined Bruton\'s tyrosine kinase (BTK) and proteasome inhibition merits further evaluation in WM.

Lymphoblastic lymphoma is a rare and aggressive form of non-Hodgkin lymphoma (NHL). The tumor can derive from T-cell or B-cell and is clinically similar to acute lymphoblastic leukemia with minimal to no bone marrow involvement distinguishing the two. We present a rare case of lymphoblastic lymphoma originating from the paranasal sinuses. A 40-year-old male presented to the emergency department and was diagnosed with right-sided acute sinusitis complicated by pre-septal cellulitis. After failing medical management, he underwent endoscopic sinus surgery. Pathologic analysis of nasal contents stained for CD79a, CD34, and PAX5, suggesting a B-cell lymphoblastic lymphoma (B-LBL). He was referred to hematology-oncology and was treated with hyperfractionated cyclophosphamide, vincristine, Adriamycin, dexamethasone (Hyper-CVAD). Short-term follow-up has thus far demonstrated near-complete resolution of the tumor. Non-Hodgkin lymphomas of the paranasal sinuses are rare, and B-cell lymphoblastic lymphomas comprise just 0.3% of adults with NHL. Immunohistochemical phenotyping for B-LBL is typically positive for B-cell markers CD19, CD20, CD22 and CD79a. Classic treatment involves a chemotherapy regimen of Hyper-CVAD with an overall survival rate of 66%. B-cell lymphoblastic lymphoma is rarely reported in the paranasal sinuses. A thorough history and physical exam along with a complete workup, including biopsies with histopathological and immunohistochemical analysis, needs to be obtained. Little is known about its prognosis when the primary site is within the paranasal sinuses, and therefore, patients need prompt and aggressive treatment when the diagnosis is made.

Background Diffuse large B-cell lymphoma (DLBCL) is the most common subtype of non-Hodgkin\'s lymphoma with a five-year survival of 60%-70% with chemoimmunotherapy consisting of the R-CHOP combination (rituximab, cyclophosphamide, vincristine, doxorubicin, and prednisone), with a relapse/refractory rate of 20-50%. Salvage therapy with HDT-ASCT is the treatment of choice for patients with relapsed/refractory disease with a success rate of 50%-60%. Patients who do not respond to the first salvage regimen or who relapsed after the first salvage regimen, with or without high-dose chemotherapy (HDT)-autologous stem cell transplantation (ASCT), have poor overall responses and survival and should be offered novel therapies. The objective of our study was to evaluate responses to second salvage, gemcitabine-based therapy with or without HDT-ASCT in a resource-limited setting. Materials and methods This was a retrospective study, including 55 patients aged >18 years, diagnosed with DLBCL and having received gemcitabine-based second salvage chemotherapy. Results The median age was 34 years, only one patient achieved progression-free survival (PFS) of >12 months with ORR of 27% to two cycles of gemcitabine-based combination, two years PFS and OS of 9.6% and 34%, respectively, and a median PFS and OS of four months and 13 months, respectively. Conclusion DLBCL patients, refractory to first-line and first salvage chemotherapy, should be considered for novel therapies or opt for palliative care rather than second salvage chemotherapy and HDT-ASCT, which results in poor overall response and significant toxicities.