title: [Successful treatment with rituximab of a patient with coincident acquired hemophilia A and thrombotic thrombocytopenic purpura].
abstract: A 66-year-old man was admitted for oral hemorrhage, purpura, and APTT prolongation. Factor VIII (FVIII) activity was decreased, due to the presence of FVIII inhibitor. He was diagnosed with acquired hemophilia A (AHA) and treated with prednisolone. Eight months later, the FVIII inhibitor titer again increased. Upon readmission, thrombocytopenia and autoimmune hemolytic anemia were found. We suspected Evans syndrome accompanied by AHA, and we treated the patient with IVIG. However, his platelet count did not increase. Speech disturbance and delirium were observed from the 12th day of hospitalization. He was subsequently diagnosed with thrombotic thrombocytopenic purpura (TTP) because ADAMTS13 inhibitor was detected, causing a decrease in ADAMTS13 activity. We initiated plasma exchange (PE) and steroid-pulse therapy. After PE for 3 days, laboratory test results and psychiatric symptoms showed dramatic improvement. However, after a 2-day period without PE, the patient's platelet count decreased markedly. Therefore, we administered rituximab to eliminate these inhibitors. His platelet count recovered rapidly, and we were able to gradually wean the patient from PE. After two additional administrations of rituximab, neither inhibitor was detected. To date, the patient has remained in complete remission for approximately 3 years.
authors.affiliation: Department of Hematology/Oncology, Okayama City Hospital.
authors.forename: Kazuhiko
authors.initials: K
authors.lastname: Yamamoto
authors.forename: Yasuhisa
authors.initials: Y
authors.lastname: Sandou
authors.forename: Masami
authors.initials: M
authors.lastname: Niiya
authors.forename: Yuuki
authors.initials: Y
authors.lastname: Osada
authors.forename: Kazuhiro
authors.initials: K
authors.lastname: Ikeuchi
authors.forename: Yoshikazu
authors.initials: Y
authors.lastname: Yamamoto
authors.forename: Yasuhiro
authors.initials: Y
authors.lastname: Shiote
authors.forename: Masanori
authors.initials: M
authors.lastname: Makita
authors.forename: Kenji
authors.initials: K
authors.lastname: Imajo
mesh_terms: D000368:Aged; D005169:Factor VIII / Q000378:metabolism; D006467:Hemophilia A / Q000150:complications / Q000188:drug therapy*; D006801:Humans; D008297:Male; D011697:Purpura, Thrombotic Thrombocytopenic / Q000150:complications / Q000188:drug therapy*; D000069283:Rituximab / Q000627:therapeutic use*; D016896:Treatment Outcome
chemical_list: D000069283:Rituximab; C078147:F8 protein, human; D005169:Factor VIII