Guizhou University of Traditional Chinese Medicine, Guiyang 550002, Guizhou, China.
Guizhou Provincial People's Hospital, Guiyang 550002, Guizhou, China.
Medicine (Baltimore). 2024 May 10;103(19):e38103. 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.
血栓性血小板减少性紫癜(TTP)是一种罕见的血栓性微血管病,由血管性血友病因子裂解蛋白酶(ADAMTS13)活性降低引起,可能危及生命。本病例研究中的患者还同时患有干燥综合征和肾功能损害,表现出多种症状,治疗极具挑战性。
一名 25 岁的产后女性,在剖宫产术后 8 天因意识淡漠超过 1 天就诊。
血小板、血红蛋白、肌酐和 ADAMTS13 水平显著降低。经眼科会诊,右眼视网膜出血,双眼干眼症。
诊断为 TTP 合并干燥综合征和肾功能损害,给予多次血浆置换联合利妥昔单抗治疗。
经治疗和随访期间,患者血小板计数和出血症状明显改善。
TTP 死亡率高,合并干燥综合征和肾功能损害时治疗更具挑战性。但给予标准血浆置换联合利妥昔单抗治疗后,治疗效果良好。