Autoimmune conditions can occur in a temporary relationship with any malignant lymphoma. In many instances, treatment at diagnosis is not required, but symptomatic autoimmune conditions represent an indication for treatment, particularly in chronic lymphoproliferative diseases. Treatment is selected depending on the predominant condition: autoimmune disease (immunosuppression) or lymphoma (antilymphoma therapy). Steroids and anti-CD20 antibodies are effective against both conditions and may suppress the autoimmune complication for a prolonged period. The efficacy of B-cell receptor inhibitors has provided us with novel insights into the pathophysiology of antibody-producing B cells. Screening for underlying autoimmune conditions is part of the lymphoma workup, because other drugs, such as immunomodulators and checkpoint inhibitors, should be avoided or used with caution. In this article, we discuss diagnostic challenges and treatment approaches for different situations involving lymphomas and autoimmune cytopenias.
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