Thrombotic Thrombocytopenic Purpura with Severe ADAMTS-13 Deficiency in a Patient with Antiphospholipid Antibodies and Charcot-Marie-Tooth Disease
スポンサーリンク
概要
- 論文の詳細を見る
A 26-year-old woman with a history of mild mental retardation, Charcot-Marie-Tooth disease (CMT) and idiopathic thrombocytopenic purpura developed severe thrombocytopenia with Coombs-negative hemolytic anemia. Magnetic resonance imaging revealed a fresh cerebral infarction in the left precentral gyrus. ADAMTS-13 deficiency caused by an inhibitor and anti-cardiolipin antibodies were detected in the blood. After treatment with prednisolone and fresh frozen plasma, ADAMTS-13 activity was normalized, the ADAMTS-13 inhibitor had disappeared and the thrombocytopenia with a bleeding tendency was improved. To our knowledge, this is the first case of thrombotic thrombocytopenic purpura caused by ADAMTS-13 deficiency associated with antiphospholipid antibodies and CMT.
著者
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Sawada Kenichi
Department Of Hematology Nephrology And Rheumatology Akita University Graduate School Of Medicine
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Masai Rie
Department Of Hematology Nephrology And Rheumatology Akita University Graduate School Of Medicine
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Takahashi Naoto
Department Of Anesthesiology University Of California San Diego School Of Medicine
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Saitoh Hirobumi
Department Of Hematology Nephrology And Rheumatology Akita University Graduate School Of Medicine
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Wakui Hideki
Department of Hematology, Nephrology, and Rheumatology, Akita University Graduate School of Medicine
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Sato Ryuta
Department of Hematology, Nephrology, and Rheumatology, Akita University Graduate School of Medicine, Japan
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Kosaka Mayu
Department of Hematology, Nephrology, and Rheumatology, Akita University Graduate School of Medicine, Japan
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Ito Mitsugu
Department of Hematology, Nephrology, and Rheumatology, Akita University Graduate School of Medicine, Japan
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