A case of systemic lupus erythematosus having concurrent Evans syndrome and acquired thrombotic thrombocytopenic purpura

Abstract

An 18-year-old Japanese woman with systemic lupus erythematosus (SLE) experienced dyspnoea, headache, tinnitus, and purpura for two weeks and was admitted to our hospital. The patient had been diagnosed with SLE and secondary immune thrombocytopenia eight years before and treated with high-dose prednisolone (PSL) and mycophenolate mofetil. Since the blood test on admission showed haemolytic anaemia with a positive direct Coombs test and anti-glycoprotein IIb/IIIa antibodies, the patient was initially diagnosed with Evans syndrome (ES). The patient was treated with pulse intravenous methylprednisolone followed by 45 mg/day prednisolone; however, the patient’s platelet count did not normalise. Based on a low level of a disintegrin-like metalloproteinase with thrombospondin type 1 motif 13 (ADAMTS-13) activity and a high level of ADAMTS-13 inhibitors, a diagnosis of acquired thrombotic thrombocytopenic purpura (TTP) was confirmed. After undergoing therapeutic plasma exchange for six consecutive days, the patient’s platelet count recovered rapidly. Although concurrent acquired TTP and ES have not been reported previously, the findings from this case highlight the importance of measuring ADAMTS-13 activity and inhibitors to rule out acquired TTP, especially when ES is refractory to glucocorticoids.