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.
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.
Katsumi
Akira
A
Department of Hematology and Oncology, Nagoya University Graduate School of Medicine, Nagoya, Japan. katsumi@med.nagoya-u.ac.jp
Matsushita
Tadashi
T
Hirashima
Kanji
K
Iwasaki
Toshihiro
T
Adachi
Tatsuya
T
Yamamoto
Koji
K
Kojima
Tetsuhito
T
Takamatsu
Junki
J
Saito
Hidehiko
H
Naoe
Tomoki
T
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
D019774:Blood Coagulation Factor Inhibitors; D001779:Blood Coagulation Factors; C065655:anti-inhibitor coagulant complex