[Effective treatment with rituximab for primary thrombotic thrombocytopenic purpura complicated with multiple cerebral infarctions].

ABSTRACT

A 22-year-old woman was diagnosed with thrombotic thrombocytopenic purpura (TTP). She had a high fever and disorientation without renal dysfunction. She immediately underwent plasma exchange and prednisolone treatment, but they proved ineffective. She subsequently suffered from left major cerebral infarction with right-side hemiplegia. Therefore, 375 mg/m(2) of rituximab was administered weekly from day 14 with informed consent. Immediate improvements were noted in not only the hematological and biochemical parameters such as platelet count, hemoglobin level, rate of fragmented red cells, and serum LDH level but also the neurological symptoms and MRI findings. The universal histopathologic findings of TTP are characterized by hyaline thrombi formed by the aggregation of platelets, mostly in small arterioles and capillaries. Therefore, abnormal findings are rarely detected by imaging modalities such as CT and MRI. Moreover, TTP with major stroke is an extremely rare occurrence. In conclusion, we present a patient with refractory TTP with major cerebral infarction, who was effectively treated with rituximab.