Successful treatment of refractory thrombotic thrombocytopenic purpura with cyclosporine; a case report

Abstract

Thrombotic thrombocytopenic purpura (TTP) is a rare and fetal disease of the blood coagulation system. The treatment of choice for TTP is plasma exchange. There are some different medical remedies for refractory cases with their own complications. The following case is the report of a refractory TTP, who responded to cyclosporine as a newer way of medical treatment. A 42-year-old woman with systemic lupus erythematosus (SLE) was admitted because of hypermenorrhea, hematuria and purpura. She was involved with acute renal failure, fever and seizure in the course of her admission. Fragmented RBC was detected in the peripheral blood smear. Lactate dehydrogenase (LDH) raised up to 1500 U/L. ADAMTS13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13) level was reduced and von Willebrand factor-cleaving protease (ADAMTS13) antibodies were elevated significantly. She was undergone plasma exchange in order to manage TTP. Additionally, she received methylprednisolone for the treatment of TTP. Despite initial response to plasma exchange and methylprednisolone, the patient did not improve. She received cyclosporine for the treatment of refractory TTP. Her clinical and laboratory abnormalities improved significantly with cyclosporine and this response was durable. We concluded that cyclosporine can be an effective treatment in refractory TTP and SLE.

Keywords: Thrombotic thrombocytopenia purpura, Cyclosporine, Systemic lupus erythematosus, Platelet, Thrombocytopenia, Plasmapheresis