2010 Volume 17 Issue 4 Pages 513-518
We experienced 2 cases of refractory thrombotic thrombocytopenic purpura (TTP) responding to neither plasma exchange (PE) nor rituximab but successfully treated with combination chemotherapy. Case 1: A 49-year-old woman was referred to our hospital with severe anemia (Hb 4.8 g/dl) and thrombocytopenia (platelet 1.1×104 /μl). The diagnosis of TTP was established based on psychoneurotic symptoms and fever that developed after hospitalization. Treatments with PE, steroid plus rituximab, vincristine monotherapy, and cyclophosphamide monotherapy were successively performed, but without clinical benefit. After these treatments proved unsuccessful, CHOP (cyclophosphamide, adriamycin, vincristine, and prednisolone) therapy led to remission of disease. Case 2: A 64-year-old man was referred to our hospital with thrombocytopenia (platelet 0.9×104 /μl). Progressive psychoneurotic symptoms, hemolytic anemia, thrombocytopenia, and fever led to the diagnosis of TTP. A disintegrin-like and metalloprotease with thrombospondin type I motif 13 (ADAMTS13) activity was below 0.5%, and an inhibitor of ADAMTS13 was detected. Treatment with PE and rituximab was successively performed without clinical benefit. After these treatments proved unsuccessful, COP (cyclophosphamide, vincristine, and prednisolone) therapy led to remission of disease. Intensive combination chemotherapy appears to be effective in treating TTP refractory to both PE and rituximab therapy.
