Rituximab for warm‐type idiopathic autoimmune hemolytic anemia: a retrospective study of 11 adult patients

Abstract

Warm-type idiopathic autoimmune hemolytic anemia (AIHA) is a relatively common hematologic disorder resulting from autoantibody production against red blood cells. Steroids represent the first-line therapeutic option, and immunosuppressive agents as well as splenectomy are used for refractory cases. Recently, the anti-CD20 monoclonal antibody rituximab has been shown to control autoimmune hemolysis in patients with refractory chronic disease. We report results from a retrospective analysis of 11 adult patients receiving rituximab for steroid-refractory AIHA of the warm type at a mean age of 55 yr (range 23-81 yr). All patients were given methyl-prednisolone as first-line treatment and some of them also received azathioprine and intravenous high-dose immunoglobulins. One patient underwent splenectomy. All patients were considered refractory to steroids and/or immunosuppressive drugs and all were then given weekly rituximab (375 mg/m(2)) for four consecutive weeks. An increase in hemoglobin (Hgb) levels in response to rituximab, with a mean increment of 3.3 g/dL (95% CI 2.1-4.4), was observed in all cases. Four patients required packed red cell transfusions before starting rituximab and all became transfusion-free. At a mean follow-up of 604 d (range 30-2884 d) since the treatment of AIHA with rituximab, all patients are alive, eight (73%) of them in complete remission (CR) and three (27%) in partial remission (PR). A moderate hemolysis still persisted in six (54%) patients. In conclusion, our experience clearly demonstrates that anti-CD20 monoclonal antibody rituximab is an effective and safe alternative treatment option for idiopathic AIHA, in particular, for steroid-refractory disease.

Rituximab in the treatment of autoimmune haematological disorders

“…Further investigations are needed to determine whether rituximab maintenance therapy prolongs remission duration in AIHA. In addition to the data reported above showing that patients can respond to re-treatment with rituxumab, preliminary data in small series of AIHA and CAD patients show that durable responses can also be achieved after extended rituximab treatment (Quartier et al, 2001;D'Arena et al, 2006D'Arena et al, , 2007Schöllkopf et al, 2006). In addition, a case report has described a patient with mantle cell lymphoma who developed acquired AIHA that was successfully treated with rituximab …”

“…Responses were observed both in patients with idiopathic AIHA (Quartier et al, 2001;Shanafelt et al, 2003;Zecca et al, 2003;Narat et al, 2005;Berentsen et al, 2006;Schöllkopf et al, 2006;D'Arena et al, 2007) and in those with AIHA secondary to a range of conditions, including bone marrow transplant (Quartier et al, 2001;Zecca et al, 2003), autoimmune disorders (Shanafelt et al, 2003;Zecca et al, 2003), chronic lymphocytic leukaemia (Gupta et al, 2002;Trapè et al, 2003;Zaja et al, 2003b;Narat et al, 2005;D'Arena et al, 2006) and other lymphoproliferative disorders (Trapè et al, 2003;Narat et al, 2005;Schöllkopf et al, 2006). Patients with warm AIHA responded well to rituximab treatment regardless of prior therapy (Quartier et al, 2001;Gupta et al, 2002;Shanafelt et al, 2003;Trapè et al, 2003;Zaja et al, 2003b;Zecca et al, 2003;Narat et al, 2005;D'Arena et al, 2006).…”

“…An overview of studies employing rituximab in AIHA demonstrated that the speed with which AIHA patients responded to rituximab treatment varied considerably, with some patients responding very quickly and others taking weeks or even months to achieve their maximum response (Gupta et al, 2002;Zaja et al, 2003b;Zecca et al, 2003;Berentsen et al, 2004;Schöllkopf et al, 2006;D'Arena et al, 2007). Responses were observed both in patients with idiopathic AIHA (Quartier et al, 2001;Shanafelt et al, 2003;Zecca et al, 2003;Narat et al, 2005;Berentsen et al, 2006;Schöllkopf et al, 2006;D'Arena et al, 2007) and in those with AIHA secondary to a range of conditions, including bone marrow transplant (Quartier et al, 2001;Zecca et al, 2003), autoimmune disorders (Shanafelt et al, 2003;Zecca et al, 2003), chronic lymphocytic leukaemia (Gupta et al, 2002;Trapè et al, 2003;Zaja et al, 2003b;Narat et al, 2005;D'Arena et al, 2006) and other lymphoproliferative disorders (Trapè et al, 2003;Narat et al, 2005;Schöllkopf et al, 2006).…”

Activity and safety profile of low-dose rituximab for the treatment of autoimmune cytopenias in adults

“…Duration of response was considered from the day of the initial infusion to the first time of relapse (platelet count <30×10 Criteria for CR in AIHA were resolution of both anemia (Hb >12g/dL) and signs of hemolysis off all therapy for at least 4 weeks after rituximab treatment. 4 A PR was defined as a stable increase in hemoglobin level of at least 2 g/dL and discontinuation of concomitant therapy.…”

Efficacy of rituximab in autoimmune hemolytic anemia associated with splenic marginal zone lymphoma

“…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].…”