Intractable gastrointestinal bleeding from angiodysplasia in a patient of Bernard-Soulier syndrome--report of a case--.

نویسندگان

  • Riki Okita
  • Jun Hihara
  • Kazuo Konishi
  • Akihiko Osaki
  • Kazuhiro Yoshida
  • Yoshiyuki Yamaguchi
  • Tetsuya Toge
  • Kingo Fujimura
  • Takashi Iwata
  • Akihiro Sawamura
چکیده

We herein report a case of gastrointestinal bleeding induced by angiodysplasia of the cecum in a case of Bernard-Soulier syndrome with recurrent breast cancer. In spite of endoscopic hemostatic therapy and interventional embolization, she had repeated massive bleeding from the cecal lesion. In addition, she had chronic hepatitis C and progressive liver tumors metastasized from breast cancer, and she finally died of hepatic failure. There are four case reports in the literature describing Bernard-Soulier syndrome with gastrointestinal bleeding angiodysplasia. The reported cases benefited from hormonal, endoscopical hemostasis and surgical therapy. In our case, because of her terminal metastatic breast cancer, only conservative treatments were administered after the third bleed. Both the endoscopic hemostatic method and interventional embolization showed only a temporary effect. Surgical treatment should be considered if the general condition can tolerate a surgical procedure.

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

منابع مشابه

Bernard-Soulier syndrome or idiopathic thrombocytopenic purpura: A case series

Background: Bernard-Soulier syndrome (BSS) is a rare, autosomal recessive platelet function disorder which is commonly mistaken for idiopathic thrombocytopenic purpura (ITP).The report includes seven cases of BSS that have been diagnosed and treated as ITP for a long time. Methods: Between 2006 and 2016, data of seven BSS patients who have long been diagnosed and treated as ITP were collected ...

متن کامل

Quantitative Immunophemotyping of Platelet Surface Glycoproteins among Iranian Patients with Bernard-Soulier Syndrome

Background: Bernard-Soulier syndrome is a rare inherited bleeding disease caused by quantitative or qualitative defect of GPIb/IX/V, a platelet complex that binds the Von Willebrand factor. The expression of GPIb-IX-V complex can be evaluated by flow cytometry and confirmed by the absence of ristocetin-induced platelet aggregation in platelet-rich plasma. The main aim of the present study was t...

متن کامل

شناسایی 5 جهش جدید در ژن گلیکوپروتئین Ibα پلاکت در بیماران برنارد- سولیر ایران

    Background & Aim: Bernard-Soulier syndrome (B.S.S) is a rare hereditary bleeding disorder due to molecular defects of platelet GPIb–IX–V. The GPIb-IX-V complex is composed of four chains of GPIbα, GPIbβ, GPIX and GPV.  The largest chain of this complex is GPIbα and is responsible for binding to ligand and most of identified mutations belong to this glycoprotein.  The aim of  this  study was...

متن کامل

Extensive Blue Rubber Bleb Nevus Syndrome with Multiple Gastrointestinal Venous Malformations: A Case Report

Blue rubber bleb nevus syndrome is a sporadic disease with widely distributed dark blue papules and nodules and soft skin-colored compressible protuberances (rubber blebs) as well as large vascular malformations. Gasterointestinal lesions are documented by upper endoscopy and colonoscopy; hemorrhages from these lesions create irondeficinecy anemia. Other sites of visceral involvement are less c...

متن کامل

Aortic Valve Replacement for the Management of Heyde Syndrome: A Case Report.

Heyde syndrome describes the triad of aortic stenosis, acquired coagulopathy, and anemia due to bleeding from intestinal angiodysplasia. An 87-year-old man with iron deficiency anemia due to melena was admitted to our hospital. On examination, a systolic murmur was heard and echocardiography confirmed the presence of aortic stenosis. Esophagogastroduodenoscopy and colonoscopy were unremarkable....

متن کامل

ذخیره در منابع من


  با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

عنوان ژورنال:
  • Hiroshima journal of medical sciences

دوره 54 4  شماره 

صفحات  -

تاریخ انتشار 2005