■患有干燥综合征(SS)的患者,免疫介导的血栓性血小板减少性紫癜(ITTP),据报道,后部可逆性脑病综合征(PRES),以及所有已发表的血栓性血小板减少性紫癜(TTP)病例,PRES,和SS进行了检索和分析。对患者的临床资料和治疗程序进行了讨论。
■一名45岁的中国女性因头痛和血小板计数低而住院。她之前曾到当地医院就诊,有7个月的上腹部不适和厌食症病史,诊断为SS和ITTP。入院后实验室检查显示血小板(PLT)为13*10^9/L,6%的红细胞(RBC)碎片,ADAMTS13活性<0.2%,88.3U/mL的抗ADAMTS13IgG。脑磁共振成像(MRI)显示左额叶皮层和双侧顶叶颞叶皮层中的回旋状扩散受限以及T2-FLAIR信号增加。她被诊断出患有党卫军,ITTP和PRES,并接受了甲基强的松龙的治疗,环孢菌素,血浆置换,IVIG,还有利妥昔单抗.该患者在8个月的随访期间没有经历复发。
■ITTP和PRES是SS的罕见表现。在怀疑或确认诊断为ITTP后,应立即进行血浆置换和免疫抑制治疗.我们建议利妥昔单抗可能对SS联合ITTP和PRES具有额外的治疗价值。
UNASSIGNED: A patient with Sjögren\'s syndrome (SS), immune-mediated thrombotic thrombocytopenic purpura (ITTP), and posterior reversible encephalopathy syndrome (PRES) was reported, and all published cases with thrombotic thrombocytopenic purpura (TTP), PRES, and SS were retrieved and analysed. The patient\'s clinical data and treatment procedure have been discussed.
UNASSIGNED: A 45-year-old Chinese female was hospitalized with headache and low platelet count. She had previously presented to a local hospital with a 7-month history of epigastric discomfort and anorexia, and was diagnosed with SS and ITTP. Laboratory investigations after admission showed platelet (PLT) of 13*10^9/L, red blood cell (RBC) fragments of 6 %, ADAMTS13 Activity<0.2 %, anti-ADAMTS13 IgG of 88.3U/mL. Brain magnetic resonance imaging (MRI) showed gyriform restricted diffusion along with increased T2-FLAIR signal in the left frontal cortex and bilateral parietal temporal cortex. She was diagnosed with SS, ITTP and PRES, and received the treatment of methylprednisolone, cyclosporine, plasma exchange, IVIG, and rituximab. This patient did not experience the recurrence during the 8-month follow-up period.
UNASSIGNED: ITTP and PRES are rare manifestations of SS. After a suspected or confirmed diagnosis of ITTP, plasma exchange and immunosuppressive therapy should be immediately administered. We suggest that rituximab could have additional therapeutic value for SS combined with ITTP and PRES.