Paraneoplastic Evans Syndrome Associated with Serous Ovarian Carcinoma

Abstract Evans Syndrome (ES) is a rare condition defined by the co-occurrence of two or more immune cytopenias, most frequently Autoimmune Hemolytic Anemia (AIHA) and Immune Thrombocytopenia (ITP). This condition is associated with a wide range of disease processes including infectious, autoimmune, or malignant etiologies. Paraneoplastic Evans syndrome is most often associated with hematological malignancies such as Chronic Lymphocytic Leukemia (CLL) or Non-Hodgkin Lymphoma (NHL), and while exceedingly rare, ES has also been reported in solid tumors. ES is considered more difficult to treat than isolated ITP or AIHA, with higher rates of treatment failure and disease recurrence. Corticosteroids are the cornerstone of therapy, but other treatment options including intravenous immunoglobulins, immunosuppressants, and hematopoietic stem cell transplantation have also been used. In this article, we report a case of a seventy-one-year-old female who had undiagnosed serous ovarian carcinoma with paraneoplatic Evans syndrome as the initial presentation. She developed signs of elevated intracranial pressure including headache, nausea and vomiting at home and was found to have life-threatening intracranial hemorrhage from ITP, in addition to critical anemia from AIHA. She responded well to treatment and survived the episode, after which her ES was controlled with chronic low-dose steroids. Extensive diagnostic workups for secondary causes of ES were unrevealing at the time, although a solid right adnexal mass was incidentally found. The lesion was not urgently investigated and periodic surveillance was preferred over prompt surgical resection. A few months later she was found to have extensive metastases from high-grade serous ovarian carcinoma and died shortly after chemotherapy was initiated. Her missed diagnosis can be attributed to a general lack of awareness of the association between ES and solid tumors. She succumbed to the cancer which, if detected early, was arguably curable. In light of this tragedy, it is our intention to raise awareness of this rare disorder and its association with uncommon etiologies.