Active Hepatitis C Leading to Refractory Thrombotic Thrombocytopenic Purpura – A Dubious Association and The Challenges Faced in Management.

Abstract

Acquired thrombotic thrombocytopenic purpura is a combination of thrombocytopenia with microangiopathic hemolytic anemia. A 62-year-old female was transferred from an outside hospital for rapidly worsening mental status and severe thrombocytopenia. Laboratory studies were significant for reduced hemoglobin and platelet count along with raised blood urea nitrogen, creatinine, and serum lactate dehydrogenase levels. Peripheral smear showed numerous schistocytes and further testing showed low ADAMTS13 activity, high ADAMTS13 inhibitor, and positive hepatitis C virus antibody with a high hepatitis C virus ribonucleic acid (RNA) load. The patient was diagnosed with acquired thrombotic thrombocytopenic purpura and started on plasma exchange and steroids. Since no response was achieved until day four of treatment, weekly rituximab was initiated. After the initial two doses of rituximab, she showed significant improvement clinically. ADAMTS13 levels returned back to normal. Cyclosporine was added, following which platelet counts were normalized. Cyclosporine was discontinued, plasma exchange and steroids were slowly tapered off. Follow-up visits showed that the patient is off treatment and continues to be in remission and on regular treatment for hepatitis C. Acquired thrombotic thrombocytopenic purpura is a hematological emergency. Our patient remained refractory to standard therapies and required rituximab and immunosuppressive agents like cyclosporine. We describe the association of active hepatitis C with acquired thrombotic thrombocytopenic purpura that was refractory to plasma exchange, high dose steroids and rituximab. As per our knowledge, this is the first case in the literature to describe a possible association between active hepatitis C and acquired thrombotic thrombocytopenic purpura.