Relapsed/refractory acquired thrombotic thrombocytopenic purpura in a patient with Sjögren syndrome: Case report and review of the literature

RATIONALE: Thrombotic thrombocytopenic purpura (TTP) is a rare, fatal disorder which could be caused by autoimmune diseases. However, TTP secondary to Sjögren syndrome (SS) is extremely rare. PATIENT CONCERNS: A 47-year- old woman with an 8-year history of SS was admitted due to skin ecchymosis and bleeding gums. Then she gradually developed fever and headache. DIAGNOSES: Laboratory investigations suggested anemia, thrombocytopenia, increased lactic dehydrogenase, and a disintegrin-like metalloproteinase with thrombospondin motif type 1 member 13 (ADAMTS13) activity deficiency with high inhibitor titers. Acquired TTP was thus diagnosed. INTERVENTIONS: Plasma exchange (PE) was the first choice for treatment, while glucocorticoid, cyclosporine A (CSA), rituximab, and intravenous immunoglobulin (IVIG) were used simultaneously. Bortezomib, a selective proteasome inhibitor and thereby inducing apoptosis in both B-cells and plasma cells, was added. OUTCOMES: She was discharged from the hospital and then treated with prednisone of 40 mg/d and hydroxychloroquine. The patient remained in full remission. LESSONS: We conclude that bortezomib should be considered for patients with TTP refractory to PE, steroids, and rituximab due to its efficacy and relatively favorable side effect profile.