title: Acquired hemophilia A with sigmoid colon cancer: successful treatment with rituximab followed by sigmoidectomy.
abstract: Acquired hemophilia A is a rare and potentially fatal condition of coagulopathy caused by autoantibodies against clotting factor VIII (factor VIII inhibitor). We report a case of a 63-year-old woman, who presented with a sudden onset of severe hemorrhagic tendency with exclusively prolonged activated partial thromboplastin time (APTT). She was diagnosed with acquired hemophilia A due to a decrease in factor VIII activity and a high titer of factor VIII inhibitor. Hemorrhage was well controlled by recombinant activated factor VII. Although the level of factor VIII inhibitor did not decline with prednisolone and cyclophosphamide, it became undetectable with rituximab. In parallel with controlling hemorrhage, malignancy, which may cause acquired hemophilia A, was searched for and sigmoid colon cancer was found. After the eradication of factor VIII inhibitor, surgical resection was performed uneventfully. Thereafter, acquired hemophilia A has been in complete remission without any additional therapy. The present case suggests the efficacy of rituximab for refractory acquired hemophilia A and the importance of the identification of underlying diseases that can cause acquired hemophilia A.
authors.affiliation: Department of Hematology and Rheumatology, Tohoku University Hospital, 1-1 Seiryo-machi, Aoba-ku, Sendai, 980-8574, Japan.
authors.forename: Satoshi
authors.initials: S
authors.lastname: Ichikawa
authors.affiliation: Department of Hematology and Rheumatology, Tohoku University Hospital, 1-1 Seiryo-machi, Aoba-ku, Sendai, 980-8574, Japan.
authors.forename: Katsura
authors.initials: K
authors.lastname: Kohata
authors.affiliation: Department of Hematology and Rheumatology, Tohoku University Hospital, 1-1 Seiryo-machi, Aoba-ku, Sendai, 980-8574, Japan.
authors.forename: Yoko
authors.initials: Y
authors.lastname: Okitsu
authors.affiliation: Department of Hematology and Rheumatology, Tohoku University Hospital, 1-1 Seiryo-machi, Aoba-ku, Sendai, 980-8574, Japan.
authors.forename: Makiko
authors.initials: M
authors.lastname: Suzuki
authors.affiliation: Department of Hematology and Rheumatology, Tohoku University Hospital, 1-1 Seiryo-machi, Aoba-ku, Sendai, 980-8574, Japan.
authors.forename: Shinji
authors.initials: S
authors.lastname: Nakajima
authors.affiliation: Department of Hematology and Rheumatology, Tohoku University Hospital, 1-1 Seiryo-machi, Aoba-ku, Sendai, 980-8574, Japan.
authors.forename: Minami F
authors.initials: MF
authors.lastname: Yamada
authors.affiliation: Department of Hematology and Rheumatology, Tohoku University Hospital, 1-1 Seiryo-machi, Aoba-ku, Sendai, 980-8574, Japan.
authors.forename: Yasushi
authors.initials: Y
authors.lastname: Onishi
authors.affiliation: Department of Hematology and Rheumatology, Tohoku University Hospital, 1-1 Seiryo-machi, Aoba-ku, Sendai, 980-8574, Japan.
authors.forename: Joji
authors.initials: J
authors.lastname: Yamamoto
authors.affiliation: Department of Hematology and Rheumatology, Tohoku University Hospital, 1-1 Seiryo-machi, Aoba-ku, Sendai, 980-8574, Japan.
authors.forename: Sozo
authors.initials: S
authors.lastname: Suzuki
authors.affiliation: Department of Hematology and Rheumatology, Tohoku University Hospital, 1-1 Seiryo-machi, Aoba-ku, Sendai, 980-8574, Japan.
authors.forename: Kenichi
authors.initials: K
authors.lastname: Ishizawa
authors.affiliation: Department of Hematology and Rheumatology, Tohoku University Hospital, 1-1 Seiryo-machi, Aoba-ku, Sendai, 980-8574, Japan.
authors.forename: Junichi
authors.initials: J
authors.lastname: Kameoka
authors.affiliation: Department of Hematology and Rheumatology, Tohoku University Hospital, 1-1 Seiryo-machi, Aoba-ku, Sendai, 980-8574, Japan. harigae@mail.tains.tohoku.ac.jp.
authors.forename: Hideo
authors.initials: H
authors.lastname: Harigae
mesh_terms: D000911:Antibodies, Monoclonal / Q000008:administration & dosage*; D058846:Antibodies, Monoclonal, Murine-Derived; D000970:Antineoplastic Agents / Q000008:administration & dosage*; D019774:Blood Coagulation Factor Inhibitors / Q000097:blood; D012809:Colon, Sigmoid / Q000601:surgery; D005169:Factor VIII / Q000032:analysis; D005260:Female; D006467:Hemophilia A / Q000097:blood / Q000188:drug therapy* / Q000209:etiology*; D006801:Humans; D008875:Middle Aged; D010314:Partial Thromboplastin Time; D000069283:Rituximab; D012811:Sigmoid Neoplasms / Q000097:blood / Q000150:complications* / Q000628:therapy*
chemical_list: D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D000970:Antineoplastic Agents; D019774:Blood Coagulation Factor Inhibitors; D000069283:Rituximab; D005169:Factor VIII