Zheng Xinglong, Pallera Arnel M, Goodnough Lawrence T, Sadler J Evan, Blinder Morey A
Washington University School of Medicine, 660 South Euclid Avenue, Box 8022, St. Louis, Missouri 63110, USA.
Ann Intern Med. 2003 Jan 21;138(2):105-8. doi: 10.7326/0003-4819-138-2-200301210-00011.
Thrombotic thrombocytopenic purpura (TTP) in adults is usually caused by autoantibody inhibitors of ADAMTS13. Treatment with plasma exchange is often effective but does not address the underlying autoimmune process.
To report the efficacy of intensive immunosuppressive therapy in refractory TTP.
Case report.
University medical center.
42-year-old woman with chronic relapsing TTP.
Immunosuppression therapy with rituximab and cyclophosphamide.
ADAMTS13 activity and inhibitors and hematologic variables for TTP.
For 19 months, the patient had relapsing thrombotic microangiopathy despite plasma exchange; splenectomy; and therapy with vincristine, prednisone, and cyclosporine. ADAMTS13 activity was low, and tests detected an IgG inhibitor that recognized the metalloprotease domain of recombinant ADAMTS13. After treatment with rituximab and cyclophosphamide, the disease remitted, ADAMTS13 levels normalized, and the inhibitor was undetectable. The patient has required no treatment for 13 months.
Intensive immunosuppressive therapy can lead to sustained clinical remission in patients with refractory autoimmune TTP.
成人血栓性血小板减少性紫癜（TTP）通常由ADAMTS13自身抗体抑制剂引起。血浆置换治疗通常有效，但无法解决潜在的自身免疫过程。
报告强化免疫抑制治疗难治性TTP的疗效。
病例报告。
大学医学中心。
一名42岁慢性复发性TTP女性。
使用利妥昔单抗和环磷酰胺进行免疫抑制治疗。
TTP的ADAMTS13活性、抑制剂和血液学变量。
19个月来，尽管进行了血浆置换、脾切除术以及长春新碱、泼尼松和环孢素治疗，患者仍有复发性血栓性微血管病。ADAMTS13活性较低，检测发现一种IgG抑制剂可识别重组ADAMTS13的金属蛋白酶结构域。使用利妥昔单抗和环磷酰胺治疗后，疾病缓解，ADAMTS13水平恢复正常，且未检测到抑制剂。患者已13个月无需治疗。
强化免疫抑制治疗可使难治性自身免疫性TTP患者实现持续临床缓解。
