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Autoimmune diseases (ADs) have close relationship with the onset of malignant lymphoma.The recent research focuses on ADs such as sjogren's syndrome (SS), rheumatoid arthritis (RA), systemic lupus erythematosus (SLE).Immune disorders might play an important role in transformation from ADs to lymphoma.Cases that diffuse large B-cell lymphoma (DLBCL) occurred after autoimmune haematological diseases and autoimmune lymphoproliferative syndrome (ALPS) was diagnosed in the course were rare.The interval of two diseases were long and the pathogenesis might be related to the abnormal function of immunity, immune inhibitors, the disorder of immune surveillance and ALPS.ALPS might be a bridge between ADs of blood system and DLBCL.It is a gradual process to develop DLBCL.It starts with immune disorders, benign lymphocytic infiltration, and at last it progresses malignant histologic variants.New therapeutic methods such as rituximab, targeting B cell activating factor (BAFF), autologous stem cell transplantation (ASCT) need to be explored to treat autoimmune haematological diseases especially refractory hematologic autoimmune cytopenias.The proper treatments might prevent ADs from malignant transformation.