Laparoscopic splenectomy for the treatment of refractory thrombotic thrombocytopenic purpura.

Abstract

Thrombotic thrombocytopenic purpura (TTP) is a serious hematologic disorder with a high rate of morbidity and mortality. We report here on the surgical and homological outcomes of laparoscopic splenectomy (LS) in a patient with refractory TTP. A 69-year-old Japanese woman was referred to our hospital because of purpura in the lower extremities. In addition to the marked thrombocytopenia, hemolytic anemia and progressive mental disorder were noted. The ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 motif 13) activity was undetectable and ADAMTS13 inhibitor was extremely increased. The diagnosis of TTP was made based on the clinical features and laboratory abnormalities. She received steroid-pulse therapy for 3days, low-dose methylprednisolone continuous infusion and plasma exchange (PE) daily for 14days. However, the patient was found to be refractory TTP to PE. The LS was performed at 15days after diagnosis. The ADAMTS13 inhibitor was not detected after LS, and in addition, the platelet count had increased to over 100,000/mm(3) on postoperative day 17. The patient remains in remission 24months after surgery. The results of our case demonstrate that LS is a safe and reasonable treatment option for patients with TTP refractory to PE.