title: Recurrent intramural hematoma of the small intestine in a severe hemophilia A patient with a high titer of factor VIII inhibitor: a case report and review of the literature.
abstract: A 17-year-old man with severe hemophilia A (factor VIII <1%) developed intermittent left upper quadrant pain. He had a high titer of factor VIII inhibitor (1024 Bethesda units/mL) and was diagnosed with intramural hematoma of the jejunum. He was managed conservatively with activated prothrombin complex concentrate (APCC), resulting in the resolution of symptoms. He developed recurrent intramural hematoma of the small intestine over the next 54 months, and was successfully treated with APCC. This case highlights a rare clinical manifestation in hemophilia patients, and also indicates the effectiveness of APCC instead of exploratory surgery for intramural hematoma. Cases of intramural hematoma of the gastrointestinal tract among hemophilia patients are also reviewed.
authors.affiliation: Department of Hematology and Oncology, Nagoya University Graduate School of Medicine, Nagoya, Japan. katsumi@med.nagoya-u.ac.jp
authors.forename: Akira
authors.initials: A
authors.lastname: Katsumi
authors.forename: Tadashi
authors.initials: T
authors.lastname: Matsushita
authors.forename: Kanji
authors.initials: K
authors.lastname: Hirashima
authors.forename: Toshihiro
authors.initials: T
authors.lastname: Iwasaki
authors.forename: Tatsuya
authors.initials: T
authors.lastname: Adachi
authors.forename: Koji
authors.initials: K
authors.lastname: Yamamoto
authors.forename: Tetsuhito
authors.initials: T
authors.lastname: Kojima
authors.forename: Junki
authors.initials: J
authors.lastname: Takamatsu
authors.forename: Hidehiko
authors.initials: H
authors.lastname: Saito
authors.forename: Tomoki
authors.initials: T
authors.lastname: Naoe
mesh_terms: D000293:Adolescent; D019774:Blood Coagulation Factor Inhibitors / Q000097:blood; D001779:Blood Coagulation Factors / Q000008:administration & dosage*; D006406:Hematoma / Q000097:blood / Q000000981:diagnostic imaging / Q000188:drug therapy* / Q000209:etiology*; D006467:Hemophilia A / Q000097:blood / Q000150:complications* / Q000000981:diagnostic imaging / Q000188:drug therapy; D006801:Humans; D007410:Intestinal Diseases / Q000097:blood / Q000000981:diagnostic imaging / Q000188:drug therapy* / Q000209:etiology*; D007583:Jejunum / Q000000981:diagnostic imaging; D008297:Male; D011859:Radiography; D013997:Time Factors
chemical_list: D019774:Blood Coagulation Factor Inhibitors; D001779:Blood Coagulation Factors; C065655:anti-inhibitor coagulant complex