Evans syndrome occurring in the setting of chronic lymphocytic leukemia (CLL) or lymphoma presents a distinct management challenge. The autoimmune cytopenias that define Evans syndrome must be addressed alongside the underlying lymphoproliferative disease.
In CLL- or lymphoma-associated Evans syndrome, the activity and stage of the clonal lymphoproliferative disorder directly inform treatment selection. Regimens must account for both the haematological malignancy and the autoimmune process simultaneously.
For patients with CLL or lymphoma-associated ES, the management of ES must obviously take into account the activity and stage of clonal LPD and some regimens combining for example, rituximab 1 cyclophosphamide and dexamethasone can be helpful for treating CLL-associated ES.