title: [Acquired thrombotic thrombocytopenic purpura after vascular prosthesis implantation for impending rupture of an abdominal aortic aneurysm].
abstract: Acquired thrombotic thrombocytopenic purpura (TTP) is caused by autoantibodies against ADAMTS13. TTP patients run a rapidly fatal course unless immediate plasma exchange (PEX) is initiated upon diagnosis. Herein, we report a 72-year-old man with TTP, which developed after he underwent artificial blood vessel replacement surgery for an abdominal aneurysm with impending rupture. In the perioperative period, the patient received several platelet transfusions for severe thrombocytopenia (minimum platelet count: 0.6×10(4)/μl). Thereafter, he was admitted to our department for rapidly progressing coma with multiple cerebral infarctions, and was transferred to the ICU. Based on the tentative diagnosis of TTP, we immediately began PEX and steroid pulse therapy. The diagnosis was confirmed thereafter by markedly reduced ADAMTS13 activity (<0.5%) and his being positive for the ADAMTS13 inhibitor. We performed PEX for five consecutive days and administered high-dose prednisolone (PSL). On the second hospital day (HD), his platelet count rose along with improvement of his consciousness level. The ADAMTS13 inhibitor was not detected on the 10th HD. TTP did not relapse and his general condition improved despite tapering of PSL. In this case, by closely monitoring ADAMTS13-related parameters and minimizing the number of plasma exchanges, the patient was able to achieve a remission without the use of boosting inhibitors.
authors.affiliation: Department of Medicine and Clinical Science, Graduate School of Medicine, Gunma University.
authors.forename: Chiaki
authors.initials: C
authors.lastname: Naito
authors.forename: Yoshiyuki
authors.initials: Y
authors.lastname: Ogawa
authors.forename: Kunio
authors.initials: K
authors.lastname: Yanagisawa
authors.forename: Takuma
authors.initials: T
authors.lastname: Ishizaki
authors.forename: Masahiro
authors.initials: M
authors.lastname: Mihara
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Handa
authors.forename: Ayami
authors.initials: A
authors.lastname: Isonishi
authors.forename: Masaki
authors.initials: M
authors.lastname: Hayakawa
authors.forename: Masanori
authors.initials: M
authors.lastname: Matsumoto
authors.forename: Yoshihisa
authors.initials: Y
authors.lastname: Nojima
mesh_terms: D000368:Aged; D017544:Aortic Aneurysm, Abdominal / Q000175:diagnosis / Q000601:surgery*; D001019:Aortic Rupture / Q000175:diagnosis / Q000601:surgery*; D019917:Blood Vessel Prosthesis Implantation / Q000009:adverse effects; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D064847:Multimodal Imaging; D011697:Purpura, Thrombotic Thrombocytopenic / Q000209:etiology / Q000628:therapy*; D014057:Tomography, X-Ray Computed