Evans syndrome associated with sterile inflammation of the central nervous system: a case report

Evans syndrome is a rare hematological disease commonly defined as Coombs-positive hemolytic anemia and immune thrombocytopenia. Pathophysiology of this disease involves decreased cluster of differentiation (CD)4+ T-helper cell counts, increased CD8+ T-suppressor cell counts, a decreased CD4/CD8 ratio, and reduced serum immunoglobulin G, M and A levels - indicating a complex immune dysregulation. Association with other autoimmune diseases has been described although involvement of the central nervous system has not been reported so far.

We here present a case of a 28-year-old woman of Turkish origin with progressive, disseminated, partly mass-forming lymphoplasmacellular infiltration (CD3+ and CD138+ cells) of the brain in association with Evans syndrome. No other central nervous system disorder could be identified on neuropathological evaluation. Although treatment with rituximab was effective to normalize erythrocyte and thrombocyte levels in her peripheral blood, it failed to dampen the inflammation in her central nervous system or prevent clinical progression. Initiation of treatment with cyclophosphamide resulted in stabilization of her central nervous system inflammation and the disease course.

The complex immune dysregulation resulting in the antibody-mediated pathologies that can be regarded as the cause of both lymphoplasmacellular encephalitis and Evans syndrome renders this association to be of clinical relevance for both neurologists and hematologists. Our experience also sheds light on the effectiveness of different treatments for both disorders and we advise clinicians to take a closer look when encountering a combination of peripheral blood diseases with affection of the central nervous system.