Autoimmune Complications of Lymphoproliferative Diseases

Peripheral autoimmune cytopenias may complicate a fraction of lymphoproliferative disorders (LPD), particularly chronic lymphocytic leukemia, non-Hodgkin B-cell lymphomas, angioimmunoblastic T-cell lymphoma and large granular lymphocytic leukemia. The most frequent complications are autoimmune hemolytic anemia and immune thrombocytopenia, followed by pure red cell aplasia, autoimmune neutropenia and other systemic/organ specific autoimmune diseases. The latter are less frequently reported and probably underdiagnosed, and include systemic lupus erythematosus, rheumatoid arthritis, Sjögren's syndrome, antiphospholipid syndrome and disorders of the hemostatic system. Therapy is mainly directed at the specific autoimmune complication when it arises in the context of a non-active LPD. However, autoimmune complications that are refractory to first line therapy usually require an LPD-directed treatment. Of note, several B-cell and T cell directed therapies that are used in LPD are also indicated or in trials for primary autoimmune cytopenias, underlying the overlapping pathogenic mechanisms between LPD and autoimmunity.