早期にrituximabを使用した難治性血栓性血小板減少性紫斑病の1例

50代，男性．数日前からの血尿にて受診し，著明な血小板減少，LDH高値，腎障害を認め，即日入院．血栓性血小板減少性紫斑病（thrombotic thrombocytopenic purpura：TTP）と考え，ステロイド薬投与，血漿交換を開始したが，第3病日に全身性痙攣，意識障害を来たし，人工呼吸器管理となった．連日血漿交換を行ったが状態改善せず，ADAMTS13活性感度以下，インヒビター高力価が判明し，第7病日からrituximabの投与を行った．連日の血漿交換施行下でようやく血小板増加，病状改善を得られ，以後，長期的に寛解を維持している．...

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Published in日本内科学会雑誌 Vol. 106; no. 1; pp. 99 - 106
Main Authors 大西, 康, 沖津, 庸子, 渡邉, 真威, 松本, 雅則, 福原, 規子, 八田, 俊介, 市川, 聡, 氷室, 真仁, 小林, 匡洋, 張替, 秀郎
Format Journal Article
LanguageJapanese
Published 一般社団法人 日本内科学会 2017
Subjects
ADAMTS13
rituximab
血栓性血小板減少性紫斑病
高LDH血症
Online AccessGet full text
ISSN0021-5384
1883-2083
DOI10.2169/naika.106.99

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Abstract 50代，男性．数日前からの血尿にて受診し，著明な血小板減少，LDH高値，腎障害を認め，即日入院．血栓性血小板減少性紫斑病（thrombotic thrombocytopenic purpura：TTP）と考え，ステロイド薬投与，血漿交換を開始したが，第3病日に全身性痙攣，意識障害を来たし，人工呼吸器管理となった．連日血漿交換を行ったが状態改善せず，ADAMTS13活性感度以下，インヒビター高力価が判明し，第7病日からrituximabの投与を行った．連日の血漿交換施行下でようやく血小板増加，病状改善を得られ，以後，長期的に寛解を維持している．
AbstractList 50代，男性．数日前からの血尿にて受診し，著明な血小板減少，LDH高値，腎障害を認め，即日入院．血栓性血小板減少性紫斑病（thrombotic thrombocytopenic purpura：TTP）と考え，ステロイド薬投与，血漿交換を開始したが，第3病日に全身性痙攣，意識障害を来たし，人工呼吸器管理となった．連日血漿交換を行ったが状態改善せず，ADAMTS13活性感度以下，インヒビター高力価が判明し，第7病日からrituximabの投与を行った．連日の血漿交換施行下でようやく血小板増加，病状改善を得られ，以後，長期的に寛解を維持している．
Author 氷室, 真仁
張替, 秀郎
小林, 匡洋
渡邉, 真威
松本, 雅則
福原, 規子
大西, 康
市川, 聡
八田, 俊介
沖津, 庸子
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  organization: 東北大学病院血液・免疫科
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References 13) Tun NM, Villani GM: Efficacy of rituximab in acute refractory or chronic relapsing non-familial idiopathic thrombotic thrombocytopenic purpura: a systematic review with pooled data analysis. J Thromb Thrombolysis 34: 347-359, 2012.
10) Coppo P, et al: Prognostic value of inhibitory anti-ADAMTS13 antibodies in adult-acquired thrombotic thrombocytopenic purpura. Br J Haematol 132: 66-74, 2006.
9) Ferrari S, et al: Prognostic value of anti-ADAMTS 13 antibody features (Ig isotype, titer, and inhibitory effect) in a cohort of 35 adult French patients undergoing a first episode of thrombotic microangiopathy with undetectable ADAMTS 13 activity. Blood 109: 2815-2822, 2007.
5) Muñiz AE, Barbee RW: Thrombotic thrombocytopenic purpura (TTP) presenting as pancreatitis. J Emerg Med 24: 407-411, 2003.
11) Scully M, et al: Guidelines on the diagnosis and management of thrombotic thrombocytopenic purpura and other thrombotic microangiopathies. Br J Haematol 158: 323-335, 2012.
12) Scully M, et al: Remission in acute refractory and relapsing thrombotic thrombocytopenic purpura following rituximab is associated with a reduction in IgG antibodies to ADAMTS-13. Br J Haematol 136: 451-461, 2007.
4) Bergmann IP, et al: Acute pancreatitis and thrombotic thrombocytopenic purpura. Eur J Med Res 13: 481-482, 2008.
3) Cohen JA, et al: Cellular source of serum lactate dehydrogenase elevation in patients with thrombotic thrombocytopenic purpura. J Clin Apher 13: 16-19, 1998.
8) 松本雅則: 血栓性血小板減少性紫斑病. 日内会誌 103: 1613-1621, 2014.
1) Matsumoto M, et al: Acquired idiopathic ADAMTS13 activity deficient thrombotic thrombocytopenic purpura in a population from Japan. PloS One 7: e33029, 2012.
14) Miyakawa Y, et al: Efficacy and safety of rituximab in Japanese patients with acquired thrombotic thrombocytopenic purpura refractory to conventional therapy. Int J Hematol 104: 228-235, 2016.
2) Sayani FA, Abrams CS: How I treat refractory thrombotic thrombocytopenic purpura. Blood 125: 3860-3867, 2015.
7) Ridolfi RL, et al: The heart and cardiac conduction system in thrombotic thrombocytopenic purpura. A clinicopathologic study of 17 autopsied patients. Ann Intern Med 91: 357-363, 1979.
6) 山本起代子, 他: 微小血栓による心筋障害が突然死の原因と考えられた血栓性血小板減少性紫斑病. 臨床血液 56: 2336-2340, 2015.
References_xml – reference: 9) Ferrari S, et al: Prognostic value of anti-ADAMTS 13 antibody features (Ig isotype, titer, and inhibitory effect) in a cohort of 35 adult French patients undergoing a first episode of thrombotic microangiopathy with undetectable ADAMTS 13 activity. Blood 109: 2815-2822, 2007.
– reference: 7) Ridolfi RL, et al: The heart and cardiac conduction system in thrombotic thrombocytopenic purpura. A clinicopathologic study of 17 autopsied patients. Ann Intern Med 91: 357-363, 1979.
– reference: 8) 松本雅則: 血栓性血小板減少性紫斑病. 日内会誌 103: 1613-1621, 2014.
– reference: 5) Muñiz AE, Barbee RW: Thrombotic thrombocytopenic purpura (TTP) presenting as pancreatitis. J Emerg Med 24: 407-411, 2003.
– reference: 4) Bergmann IP, et al: Acute pancreatitis and thrombotic thrombocytopenic purpura. Eur J Med Res 13: 481-482, 2008.
– reference: 12) Scully M, et al: Remission in acute refractory and relapsing thrombotic thrombocytopenic purpura following rituximab is associated with a reduction in IgG antibodies to ADAMTS-13. Br J Haematol 136: 451-461, 2007.
– reference: 14) Miyakawa Y, et al: Efficacy and safety of rituximab in Japanese patients with acquired thrombotic thrombocytopenic purpura refractory to conventional therapy. Int J Hematol 104: 228-235, 2016.
– reference: 10) Coppo P, et al: Prognostic value of inhibitory anti-ADAMTS13 antibodies in adult-acquired thrombotic thrombocytopenic purpura. Br J Haematol 132: 66-74, 2006.
– reference: 11) Scully M, et al: Guidelines on the diagnosis and management of thrombotic thrombocytopenic purpura and other thrombotic microangiopathies. Br J Haematol 158: 323-335, 2012.
– reference: 13) Tun NM, Villani GM: Efficacy of rituximab in acute refractory or chronic relapsing non-familial idiopathic thrombotic thrombocytopenic purpura: a systematic review with pooled data analysis. J Thromb Thrombolysis 34: 347-359, 2012.
– reference: 1) Matsumoto M, et al: Acquired idiopathic ADAMTS13 activity deficient thrombotic thrombocytopenic purpura in a population from Japan. PloS One 7: e33029, 2012.
– reference: 3) Cohen JA, et al: Cellular source of serum lactate dehydrogenase elevation in patients with thrombotic thrombocytopenic purpura. J Clin Apher 13: 16-19, 1998.
– reference: 2) Sayani FA, Abrams CS: How I treat refractory thrombotic thrombocytopenic purpura. Blood 125: 3860-3867, 2015.
– reference: 6) 山本起代子, 他: 微小血栓による心筋障害が突然死の原因と考えられた血栓性血小板減少性紫斑病. 臨床血液 56: 2336-2340, 2015.
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Snippet 50代，男性．数日前からの血尿にて受診し，著明な血小板減少，LDH高値，腎障害を認め，即日入院．血栓性血小板減少性紫斑病（thrombotic thrombocytopenic...
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StartPage 99
SubjectTerms ADAMTS13
rituximab
血栓性血小板減少性紫斑病
高LDH血症
Title 早期にrituximabを使用した難治性血栓性血小板減少性紫斑病の1例
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