In conclusion, although it should be noted that patients unsuccessfully treated with rituximab are much less likely to be reported, the analysis of the available literature suggests that this monoclonal antibody represents a valid therapeutic option in the treatment of AHA in NHL patients. Keeping in mind that a high proportion of patients relapse after an initial response to rituximab, it remains to be determined if this drug may play a role even in the maintenance. Considering both the pathogenetic mechanisms underlying AHA in NHL patients as well as the poor response rates offered by other therapeutic alternatives, we conclude that rituximab can be considered a valid strategy in NHL patients with steroid refractory AHA as well as a possible option for first-line treatment in selected cases.
In These situations, tests of the ADAMTS-13 activity seem to be the most useful for the diagnosis in TTP. Fujimura and Matsumoto categorized TTP using plasma levels of ADAMTS13 activity (<3%; severe, 3–25%; moderate, 25–50%; mild) for the purpose of distinguishing TTP from TMA. They also reported that the levels of ADAMTS-13 activity are lower in idiopathic TTP than in secondary TTP [ 22 ] . Generally, patients with autoimmune diseases tended to a have mild-to-moderate deﬁciency in ADAMTS-13 activity. In addition, Coppo et al. reported that patients with severe ADAMTS-13 deﬁciency in adult idiopathic TMA are characterized by various autoimmune manifestations, a lower platelet count, and mild renal involvement [ 23 ] .