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

• Published in: Medicine (Baltimore) 2018-10, Vol.97 (43), p.e12989-e12989
• Main Authors: Sun, Rurong, Gu, Weiying, Ma, Yingchun, Wang, Jing, Wu, Min
• Format: Article
• Language: English
• Subjects: Clinical Case Report; Diagnosis, Differential; Female; Humans; Middle Aged; Purpura, Thrombotic Thrombocytopenic - complications; Purpura, Thrombotic Thrombocytopenic - diagnosis; Purpura, Thrombotic Thrombocytopenic - therapy; Sjogren's Syndrome - complications; Sjogren's Syndrome - diagnosis; Sjogren's Syndrome - therapy
• ISSN: 0025-7974; 1536-5964
• DOI: 10.1097/MD.0000000000012989

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. 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. 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. 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. She was discharged from the hospital and then treated with prednisone of 40 mg/d and hydroxychloroquine. The patient remained in full remission. 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.