ADAMTS13 and thrombotic thrombocytopenic purpura-hemolytic uremic syndrome.
نویسندگان
چکیده
In the original description and isolation of the murine Abelson leukemia virus, we described a “massive degree of meningeal involvement” as a characteristic of the disease process.1,2 As in the Bcr/Abl murine bone marrow transduction and transplantation model, the latent period to tumor appearance was very short. We suggested that the meningeal disease could serve as a model for testing chemotherapy.
منابع مشابه
Inherited thrombotic thrombocytopenic purpura mimicking immune thrombocytopenic purpura during pregnancy: a case report
BACKGROUND Thrombotic thrombocytopenic purpura is a very rare hereditary blood deficiency disorder of ADAMTS13 (von Willebrand factor-cleaving protease) and a life-threatening thrombotic microangiopathy characterized by thrombocytopenia and microangiopathic hemolytic anemia. The deficiency in ADAMTS13 metalloprotease, which cleaves the von Willebrand factor, may be congenital or acquired. The c...
متن کاملThe association of pregnancy with thrombotic thrombocytopenic purpura-hemolytic uremic syndrome.
Thrombotic thrombocytopenic purpura-hemolytic uremic syndrome occurs more commonly in women and among women is commonly associated with pregnancy. Case series of thrombotic thrombocytopenic purpura-hemolytic uremic syndrome from 1964 to 2002 were reviewed (1) to document the reports of occurrence of thrombotic thrombocytopenic purpura-hemolytic uremic syndrome during pregnancy and (2) to search...
متن کاملIdiopathic combined, autoantibody-mediated ADAMTS-13/factor H deficiency in thrombotic thrombocytopenic purpura-hemolytic uremic syndrome in a 17-year-old woman: a case report
INTRODUCTION Thrombotic thrombocytopenic purpura-hemolytic uremic syndrome is a life-threatening condition with various etiopathogeneses. Without therapy approximately 90% of all patients die from the disease. CASE PRESENTATION We report the case of a 17-year-old Caucasian woman with widespread hematomas and headache. Due to hemolytic anemia, thrombocytopenia, and schistocytosis, thrombotic t...
متن کاملVon Willebrand factor-cleaving protease (ADAMTS13) in thrombocytopenic disorders: a severely deficient activity is specific for thrombotic thrombocytopenic purpura.
A severe deficiency in von Willebrand factor-cleaving protease (ADAMTS13) activity (< 5% that in normal plasma) has been observed in most patients with a diagnosis of thrombotic thrombocytopenic purpura (TTP) but not in those with a diagnosis of hemolytic uremic syndrome. However, ADAMTS13 deficiency has been claimed not to be specific for TTP, since it was observed in various thrombocytopenic ...
متن کاملHemolytic-Uremic Syndrome
Hemolytic-uremic syndrome (HUS) is the most common cause of acute renal failure in children and is increasingly recognized in adults.[1, 2] Thrombotic thrombocytopenic purpura (TTP), childhood HUS, and adult HUS differ in their clinical presentations, but these conditions have many common features. Gasser et al first described hemolytic-uremic syndrome (HUS) in 1955. In 1988, Wardle described h...
متن کاملBrief report Von Willebrand factor–cleaving protease (ADAMTS13) in thrombocytopenic disorders: a severely deficient activity is specific for thrombotic thrombocytopenic purpura
A severe deficiency in von Willebrand factor–cleaving protease (ADAMTS13) activity (< 5% that in normal plasma) has been observed in most patients with a diagnosis of thrombotic thrombocytopenic purpura (TTP) but not in those with a diagnosis of hemolytic uremic syndrome. However, ADAMTS13 deficiency has been claimed not to be specific for TTP, since it was observed in various thrombocytopenic ...
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ورودعنوان ژورنال:
- Blood
دوره 102 10 شماره
صفحات -
تاریخ انتشار 2003