Plasmapheresis combined with rituximab treatment of a case of thrombotic thrombocytopenic purpura with Sjögren syndrome and renal impairment: A case report

• Published in: Medicine (Baltimore) 2024-05, Vol.103 (19), p.e38103
• Main Authors: Zhang, Yongqiang, Hu, Shanshan, Deng, Yiyao, Yang, Zhi, Yuan, Jing
• Format: Article
• Language: English
• Subjects: Adult; Clinical Case Report; Combined Modality Therapy; Female; Humans; Immunologic Factors - administration & dosage; Immunologic Factors - therapeutic use; Plasmapheresis - methods; Purpura, Thrombotic Thrombocytopenic - complications; Purpura, Thrombotic Thrombocytopenic - drug therapy; Purpura, Thrombotic Thrombocytopenic - therapy; Renal Insufficiency - etiology; Renal Insufficiency - therapy; Rituximab - administration & dosage; Rituximab - therapeutic use; Sjogren's Syndrome - complications; Sjogren's Syndrome - therapy
• ISSN: 0025-7974; 1536-5964; 1536-5964
• DOI: 10.1097/MD.0000000000038103

Thrombotic thrombocytopenic purpura (TTP) is a rare thrombotic microangiopathy caused by reduced activity of the von Willebrand factor-cleaving protease (ADAMTS13), which can be life-threatening. The patient reported in this case study also had concurrent Sjögren syndrome and renal impairment, presenting multiple symptoms and posing a great challenge in treatment. A 25-year-old woman in the postpartum period visited the hospital due to indifference in consciousness for more than 1 day following cesarean section 8 days prior. Notable decreases were observed in platelets, hemoglobin, creatinine, and ADAMTS13 levels. After a consultative examination by an ophthalmologist, she was diagnosed with retinal hemorrhage in the right eye and dry eye syndrome in both eyes. Having been diagnosed with TTP with Sjögren syndrome and renal impairment, she received repeated treatments with plasmapheresis combined with rituximab. Following treatment and during the follow-up period, the patient's platelet counts and bleeding symptoms significantly improved. TTP has a high mortality rate, and when combined with Sjögren syndrome and renal impairment, it poses an even greater challenge in treatment. However, after administering standard plasmapheresis combined with rituximab treatment, the treatment outcome is favorable.