Rituximab for primary chronic cold agglutinin disease: a prospective study of 37 courses of therapy in 27 patients

Abstract

Conventional therapies for primary chronic cold agglutinin disease (CAD) are ineffective, but remissions after treatment with the anti-CD20 antibody rituximab have been described in a small, prospective trial and in some case reports. In this study we report on 37 courses of rituximab administered prospectively to 27 patients. Fourteen of 27 patients responded to their first course of rituximab, and 6 of 10 responded to retreatment. In both groups combined, responses were achieved after 20 of 37 courses, giving an overall response rate of 54%. We observed 1 complete and 19 partial responses. Two nonresponders and 3 patients who experienced relapse received second-line therapy with interferon-␣ combined with a new course of rituximab, and 1 nonresponder and 2 patients who experienced relapse achieved partial responses. Responders achieved a median increase in hemoglobin levels of 40 g/L (4 g/dL). Median time to response was 1.5 months, and median observed response duration was 11 months. We conclude that rituximab is an effective and well-tolerated therapy for CAD. Histologic and flow cytometric findings suggest that some of the effect may be mediated by mechanisms other than the elimination of clonal lymphocytes. We were unable to predict responses from the hematologic, immunologic, or histologic parameters before

Autoimmune cytopenias after umbilical cord blood transplantation in adults with hematological malignancies: a single-center experience

“…Very good responses have also been reported among patients with cold agglutinin disease. 22,23 Rituximab should therefore be considered early in the management of AIHA after UCBT. Among patients with AIT, there were three transient responses to i.v.…”

Rituximab in the treatment of autoimmune haematological disorders

“…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). Likewise, rituximab was effective in patients with CAD regardless of whether they had previously been treated with immunosuppressant drug regimens and/or corticosteroids (Berentsen et al, 2004;Schöllkopf et al, 2006). In addition, there was no difference between CAD responders and nonresponders with regard to their initial haemoglobin or serum monoclonal antibody levels, cold agglutinin titre or bone marrow j:k ratio (Berentsen 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).…”

Lower dose rituximab is active in adults patients with idiopathic thrombocytopenic purpura

“…Rituximab is an emerging new agent for the treatment of several autoimmune disorders [1][2][3][4][5][6][7][8][9] and, in particular, many reports highlighted the effect of rituximab in idiopathic thrombocytopenic purpura (ITP). [10][11][12][13][14][15][16][17][18] To date, rituximab has been administered with the same schedule proposed for Bcell lymphomas, i.e.…”