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      KCI등재 SCOPUS SCIE

      비장적출술로 호전된 불응성 혈전성 혈소판감소성 자반증 1예 = A Case of Splenectomy in a Patient with Refractory Thrombotic Thrombocytopenic Purpura

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      https://www.riss.kr/link?id=A104743254

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      다국어 초록 (Multilingual Abstract)

      The introduction of plasma exchange has significantly improved the outcome of thrombotic thrombocytopenic purpura (TTP) and the survival rate was increased from 10 to 80-90%. TTP refractory to plasma exchange therapy, however, is still a therapeutic challenge. We describe here a patient who partially responded to plasma exchange therapy, but remained dependent on plasma infusions. To discontinue plasma therapy, several attempts using agents such as rituximab, vincristine, and cyclosporine A had been tried, but all failed. After splenectomy, serum LDH and blood platelet count were normalized. Plasmapheresis were we able to discontinue after 2 weeks of splenectomy. Steroid and cyclosporine were tapered off after 3 months and 5 months after splenectomy respectively, and the patient has been staying in remission ever since. We suggest that splenectomy is a worthwhile treatment option in patients with refractory TTP.
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      The introduction of plasma exchange has significantly improved the outcome of thrombotic thrombocytopenic purpura (TTP) and the survival rate was increased from 10 to 80-90%. TTP refractory to plasma exchange therapy, however, is still a therapeutic c...

      The introduction of plasma exchange has significantly improved the outcome of thrombotic thrombocytopenic purpura (TTP) and the survival rate was increased from 10 to 80-90%. TTP refractory to plasma exchange therapy, however, is still a therapeutic challenge. We describe here a patient who partially responded to plasma exchange therapy, but remained dependent on plasma infusions. To discontinue plasma therapy, several attempts using agents such as rituximab, vincristine, and cyclosporine A had been tried, but all failed. After splenectomy, serum LDH and blood platelet count were normalized. Plasmapheresis were we able to discontinue after 2 weeks of splenectomy. Steroid and cyclosporine were tapered off after 3 months and 5 months after splenectomy respectively, and the patient has been staying in remission ever since. We suggest that splenectomy is a worthwhile treatment option in patients with refractory TTP.

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      참고문헌 (Reference)

      1 이영진, "혈장교환술에 불응한 혈전성혈소판감소성자반증에서 비장절제술 1예" 대한진단검사의학회 26 (26): 119-122, 2006

      2 Kim KH, "Von Willebrand Factor-Cleaving Protease Activity in Thrombotic Thrombocytopenic Purpura" 8 : 51-58, 2001

      3 Moake JL, "Unusually large plasma factor VIII: von Willebrand factor multimers in chronic relapsing thrombotic thrombocytopenic purpura" 307 : 1432-1435, 1982

      4 Kahwash E, "Twice daily plasma exchange in refractory thrombotic thrombocytopenic purpura" 8 : 254-257, 2004

      5 Byrnes JJ, "Treatment of thrombotic thrombocytopenic purpura with plasma" 297 : 1386-1389, 1977

      6 McCarthy LJ, "Thrombotic thrombocytopenic purpura: yesterday, today, tomorrow" 8 : 80-86, 2004

      7 Cuttner J, "Thrombotic thrombocytopenic purpura: a ten-year experience" 56 : 302-306, 1980

      8 Kremer Hovinga JA, "Splenectomy in relapsing and plasma-refractory acquired thrombotic thrombocytopenic purpura" 89 : 320-324, 2004

      9 Kappers-Klunne MC, "Splenectomy for the treatment of thrombotic thrombocytopenic purpura" 130 : 768-776, 2005

      10 Coppo P, "Réseau d'Etude des microangiopathies thrombotiques de l'Adulte : Prognostic value of inhibitory anti-ADAMTS13 antibodies in adult- acquired thrombotic thrombocytopenic purpura" 132 : 66-74, 2006

      1 이영진, "혈장교환술에 불응한 혈전성혈소판감소성자반증에서 비장절제술 1예" 대한진단검사의학회 26 (26): 119-122, 2006

      2 Kim KH, "Von Willebrand Factor-Cleaving Protease Activity in Thrombotic Thrombocytopenic Purpura" 8 : 51-58, 2001

      3 Moake JL, "Unusually large plasma factor VIII: von Willebrand factor multimers in chronic relapsing thrombotic thrombocytopenic purpura" 307 : 1432-1435, 1982

      4 Kahwash E, "Twice daily plasma exchange in refractory thrombotic thrombocytopenic purpura" 8 : 254-257, 2004

      5 Byrnes JJ, "Treatment of thrombotic thrombocytopenic purpura with plasma" 297 : 1386-1389, 1977

      6 McCarthy LJ, "Thrombotic thrombocytopenic purpura: yesterday, today, tomorrow" 8 : 80-86, 2004

      7 Cuttner J, "Thrombotic thrombocytopenic purpura: a ten-year experience" 56 : 302-306, 1980

      8 Kremer Hovinga JA, "Splenectomy in relapsing and plasma-refractory acquired thrombotic thrombocytopenic purpura" 89 : 320-324, 2004

      9 Kappers-Klunne MC, "Splenectomy for the treatment of thrombotic thrombocytopenic purpura" 130 : 768-776, 2005

      10 Coppo P, "Réseau d'Etude des microangiopathies thrombotiques de l'Adulte : Prognostic value of inhibitory anti-ADAMTS13 antibodies in adult- acquired thrombotic thrombocytopenic purpura" 132 : 66-74, 2006

      11 Aqui NA, "Role of splenectomy in patients with refractory or relapsed thrombotic thrombocytopenic purpura" 18 : 51-54, 2003

      12 George JN, "Rituximab therapy for thrombotic thrombocytopenic purpura: a proposed study of the Transfusion Medicine/Hemostasis Clinical Trials Network with a systematic review of rituximab therapy for immune- mediated disorders" 21 : 49-56, 2006

      13 Yomtovian R, "Rituximab for chronic recurring thrombotic thrombocytopenic purpura: a case report and review of the literature" 124 : 787-795, 2004

      14 Furlan M, "Partial purification and characterization of a protease from human plasma cleaving von Willebrand factor to fragments produced by in vivo proteolysis" 87 : 4223-4234, 1996

      15 Allford SL, "Haemostasis and Thrombosis Task Force, British Committee for Standards in Haematology:Guidelines on the diagnosis and management of the thrombotic microangiopathic haemolytic anaemias" 120 : 556-573, 2003

      16 Rock GA, "Comparison of plasma exchange with plasma infusion in the treatment of thrombotic thrombocytopenic purpura. Canadian Apheresis Study Group" 325 : 393-397, 1991

      17 Moschcowitz E, "An acute febrile pleiochromic anemia with hyaline thrombosis of the terminal arterioles and capillaries: an undescribed disease. 1925" 70 : 352-355, 2003

      18 Furlan M, "Acquired deficiency of von Willebrand factor-cleaving protease in a patient with thrombotic thrombocytopenic purpura" 91 : 2839-2846, 1998

      19 Yoon SY, "A Case of Splenectomy in a Patient with Chronic Relapsing Thrombotic Thrombocytopenic Purpura" 4 : 79-84, 1997

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      학술지 이력

      학술지 이력
      연월일 이력구분 이력상세 등재구분
      2023 평가예정 해외DB학술지평가 신청대상 (해외등재 학술지 평가)
      2020-01-01 평가 등재학술지 유지 (해외등재 학술지 평가) KCI등재
      2011-11-29 학술지명변경 한글명 : The Korean Journal of Nephrology -> Kidney Research and Clinical Practice
      외국어명 : 미등록 -> Kidney Research and Clinical Practice
      KCI등재
      2010-01-01 평가 등재학술지 유지 (등재유지) KCI등재
      2008-01-01 평가 등재학술지 유지 (등재유지) KCI등재
      2007-02-22 학술지명변경 한글명 : 대한신장학회지 -> The Korean Society of Nephrology KCI등재
      2007-02-22 학술지명변경 한글명 : 대한신장학회지 -> The Korean Journal of Nephrology KCI등재
      2005-01-01 평가 등재학술지 선정 (등재후보2차) KCI등재
      2004-01-01 평가 등재후보 1차 PASS (등재후보1차) KCI등재후보
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      기준연도 WOS-KCI 통합IF(2년) KCIF(2년) KCIF(3년)
      2016 0.21 0.21 0.17
      KCIF(4년) KCIF(5년) 중심성지수(3년) 즉시성지수
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