Standard treatment regimens for the management of patients with refractory splenic marginal zone lymphoma (SMZL) are currently unavailable. Here, we report a case of SMZL, which, after failing multiple therapeutics, demonstrated an impressive clinical response to combined Venetoclax and Velcade (V2), a treatment combination currently being investigated in the setting of refractory multiple myeloma. We also report a unique histopathology and mutational profile that may have important implications for the characterization and prognosis of SMZL.
Keywords: Bortezomib (Velcade); Venetoclax; chemotherapy refractory; progression without transformation; relapsed/refractory (R/R); splenic marginal zone lymphoma (SMZL).