지원사업
학술연구/단체지원/교육 등 연구자 활동을 지속하도록 DBpia가 지원하고 있어요.
커뮤니티
연구자들이 자신의 연구와 전문성을 널리 알리고, 새로운 협력의 기회를 만들 수 있는 네트워킹 공간이에요.
초록· 키워드
오류제보하기
Thrombotic thrombocytopenic purpura (TTP) is a rare blood disorder with a mortality rate of over 90% without prompt treatment. It is caused by congenital, idiopathic, or secondary diseases; idiopathic TTP is mainly associated with deficiency of ADAMTS13, a von Willebrand factor cleaving protease or ADAMTS13 inhibitors. The long-term survival rate of TTP has improved since the introduction of therapeutic plasma exchange (TPE), and the therapeutic aims have also been established. However, deciding on the end-point and appropriate treatment method requires careful assessment of clinical conditions of patients. The present study reports a case of a 33-year-old male patient with reduced ADAMTS13 activity and ADAMTS13 inhibitor, who developed symptoms after an early termination of TPE with improved symptoms, which finally improved with retreatment and additionally corticosteroid. We report our case with relevant literature review on TPE in TTP with this case.
목차
등록된 정보가 없습니다.
참고문헌 (14)
참고문헌 신청
Zheng XL / 2015 / ADAMTS13 and von Willebrand factor in thrombotic thrombocytopenic purpura / Annu Rev Med 66:211~225
McPherson RA / 2016 / Henry’s clinical diagnosis and management by laboratory methods / Elsevier :774~775
Sarode R / 2014 / Thrombotic thrombocytopenic purpura: 2012 American Society for Apheresis (ASFA) consensus conference on classification, diagnosis, management, and future research / J Clin Apher 29:148~167
Schwartz J / 2016 / Guidelines on the use of therapeutic apheresis in clinical practice-evidence-based approach from the writing committee of the American Society for Apheresis: the seventh special issue / J Clin Apher 31:149~162
Rock GA / 1991 / Comparison of plasma exchange with plasma infusion in the treatment of thrombotic thrombocytopenic purpura / N Engl J Med 325:393~397
McPherson RA / 2016 / Henry’s clinical diagnosis and management by laboratory methods / Elsevier :774~775
Sarode R / 2014 / Thrombotic thrombocytopenic purpura: 2012 American Society for Apheresis (ASFA) consensus conference on classification, diagnosis, management, and future research / J Clin Apher 29:148~167
Schwartz J / 2016 / Guidelines on the use of therapeutic apheresis in clinical practice-evidence-based approach from the writing committee of the American Society for Apheresis: the seventh special issue / J Clin Apher 31:149~162
Rock GA / 1991 / Comparison of plasma exchange with plasma infusion in the treatment of thrombotic thrombocytopenic purpura / N Engl J Med 325:393~397
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