Autoimmune cytopenia in chronic lymphocytic leukemia: diagnosis and treatment.

Abstract

Treatment response and survival of 83patients with CLL complicated by immune cytopenia (IC) were analyzed. Treatment schedules in 58medicated patients included corticosteroids; chemotherapy (COP, CHOP regimens), immunotherapy (rituximab alone), immunochemotherapy (rituximab-containing regimens- R-COP, R-CHOP). Twenty-five patients underwent splenectomy. The use of corticosteroids, as the first line of treatment, resulted in short-term remission in most patients. Chemotherapy was effective in a half of CLL patients, but duration of the remission did not exceed 32months in CLL associated with autoimmune hemolytic anemia and immune thrombocytopenia. After rituximab monotherapy (10patients) the stable remission was reached in 60% of the patients with median relapse-free survival of 40months. Rituximab containing chemotherapy (22patients) caused the long-term remission in 72% of the patients with median relapse-free survival of 76months. Splenectomy performed in 25patients with CLL complicated by IC was effective in 70% of the patients. The outcome of splenectomy depends on IC entity. The best response was registered in associated immune thrombocytopenia (median overall survival 118months), the worst- in Fisher- Evans syndrome (15months). The treatment of patients with CLL complicated by ICs should be individualized. For CLL patients without significant enlargement of lymph nodes and spleen, low lymphocytosis, associated with autoimmune hemolytic anemia or immune thrombocytopenia, the monotherapy with rituximab is optimal. In case of occurrence of autoimmune hemolytic anemia, immune thrombocytopenia or Fisher- Evans syndrome in CLL patients with enlargement of lymph nodes, spleen, significant lymphocytosis, the use of R-COP or R-CHOP schemes, 4-6courses, is the most effective. Splenectomy is indicated in patients with massive splenomegaly, the resistance to medication, recurrent relapses after adequate therapy.