Paroxysmal nocturnal haemoglobinuria and Budd-Chiari syndrome.
نویسندگان
چکیده
An 11 year old boy developed pancytopenia, haemolysis, and Budd-Chiari syndrome. The venous thrombosis extended to involve other intra-abdominal vessels before paroxysmal nocturnal haemoglobinuria was recognised as the underlying haematological abnormality. Earlier diagnosis would have made curative bone marrow transplantation a possibility.
منابع مشابه
Ultrasound-guided sternal bone marrow aspiration
Leebeek FW. Myeloproliferative disease in the pathogenesis and survival of Budd-Chiari syndrome. Haematologica 2006;91: 1712-3. 3. Valla D, Dhumeaux D, Babany G, et al. Hepatic vein thrombosis in paroxysmal nocturnal hemoglobinuria. A spectrum from asymptomatic occlusion of hepatic venules to fatal Budd-Chiari syndrome. Gastroenterology 1987;93:569-75. 4. Hillmen P, Lewis SM, Bessler M, Luzzatt...
متن کاملPositive Impact of Eculizumab Therapy on Surgery for Budd-Chiari Syndrome in a Patient with Paroxysmal Nocturnal Hemoglobinuria and a Long-Term History of Thrombosis
Paroxysmal nocturnal hemoglobinuria (PNH) is associated with severe end-organ damage and a high risk of thrombosis. Budd-Chiari syndrome, which develops after thrombotic occlusion of major hepatic blood vessels, is relatively common in PNH and has been associated with increased mortality. We report the case of a 46-year-old male with PNH who presented with Budd-Chiari syndrome associated with p...
متن کاملEculizumab in paroxysmal nocturnal hemoglobinuria with Budd-Chiari syndrome progressing despite anticoagulation
Paroxysmal nocturnal hemoglobinuria (PNH) is a progressive, life-threatening disorder characterized by chronic intravascular hemolysis caused by uncontrolled complement activation. Hepatic vein thrombosis (Budd-Chiari syndrome) is common in PNH patients. This case report describes the response to eculizumab (a humanized monoclonal antibody that inhibits terminal complement activation) in a 25-y...
متن کاملTNF-α-308 G/A Polymorphism in Egyptian Budd-Chiari Syndrome Patients
Budd-Chiari syndrome (BCS) is an uncommon condition induced by thrombotic or non-thrombotic obstruction of hepatic venous outflow. BCS most often occurs in patients with underlying thrombotic diathesis, including such myeloproliferative disorders (MPDs) as polycythemia vera and paroxysmal nocturnal hemoglobinuria, and pregnancy, oral contraceptives, tumors, chronic inflammatory diseases, clotti...
متن کاملBudd-Chiari syndrome with multiple large regenerative nodules
Budd-Chiari syndrome is defined as hepatic venous outflow tract obstruction regardless of the level of the obstruction (anywhere between the small hepatic veins to the junction of the inferior vena cava and right atrium) or the cause of obstruction. It is frequently associated with hepatomegaly, abdominal pain, ascites and hepatic dysfunction, and patients with Budd-Chiari syndrome often demons...
متن کاملذخیره 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 ناماگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید
ورودعنوان ژورنال:
- Archives of disease in childhood
دوره 72 3 شماره
صفحات -
تاریخ انتشار 1995