Autoimmune manifestations including autoimmune hemolytic
anemia (AIHA) are often described in association with
splenic marginal zone lymphoma (SMZL) [1]. Moreover,
AIHA has been recently reported as a potential risk factor
of poor outcome for SMZL patients [2]. Among the
different available therapeutic options, rituximab monotherapy has been recently shown to be extremely effective in SMZL [3]. On the other hand, this monoclonal antibody plays a key role in the treatment of steroid-refractory AIHA [4]. More specifically, although rituximab has been recently included among the possible strategies to treat AIHA secondary to chronic lymphocytic leukemia [5], very few cases describing its use in the course of SMZL-related AIHA have been described so far [6]