Search results for "Microangiopathic hemolytic anemia"
showing 5 items of 5 documents
Safety of Caplacizumab in Patients Without Documented Severe ADAMTS13 Deficiency During the HERCULES Study
2019
Background: Acquired thrombotic thrombocytopenic purpura (aTTP) is a rare, life-threatening autoimmune thrombotic microangiopathy caused by a deficiency in the activity of ADAMTS13 leading to the formation of ultra-large multimers of von Willebrand factor (vWF) and abnormal platelet adhesion in the microvasculature. aTTP requires prompt diagnosis and rapid initiation of treatment to limit the risk of negative or fatal outcomes. The clinical diagnosis of aTTP is based on thrombocytopenia and microangiopathic hemolytic anemia and is confirmed by ADAMTS13 <10%. However, the latter confirmation is not always rapidly available, and treatment is typically initiated based on the clinical diagno…
Depression and cognitive deficits as long-term consequences of thrombotic thrombocytopenic purpura
2017
BACKGROUND Thrombotic thrombocytopenic purpura (TTP) is an acute life-threatening microangiopathy with a tendency of relapse characterized by consumptive thrombocytopenia, microangiopathic hemolytic anemia, and spontaneous von Willebrand factor–induced platelet clumping leading to microthrombi. The brain is frequently affected by microthrombi leading to neurologic abnormalities of varying severity. STUDY DESIGN AND METHODS The aim of this observational cohort study was to investigate the prevalence of depression and cognitive deficits in 104 patients having survived acute TTP. TTP survivors were repeatedly assessed by means of different standardized questionnaires to evaluate depression (ID…
Two Patients With History of STEC-HUS, Posttransplant Recurrence and Complement Gene Mutations
2013
Hemolytic uremic syndrome (HUS) is a disease of microangiopathic hemolytic anemia, thrombocytopenia and acute renal failure. About 90% of cases are secondary to infections by Escherichia coli strains producing Shiga-like toxins (STEC-HUS), while 10% are associated with mutations in genes encoding proteins of complement system (aHUS). We describe two patients with a clinical history of STEC-HUS, who developed end-stage renal disease (ESRD) soon after disease onset. They received a kidney transplant but lost the graft for HUS recurrence, a complication more commonly observed in aHUS. Before planning a second renal transplantation, the two patients underwent genetic screening for aHUS-associat…
Best practices and recommendations for drug regimens and plasma exchange for immune thrombotic thrombocytopenic purpura.
2021
Introduction Thrombotic thrombocytopenic purpura (TTP) is a life-threatening thrombotic microangiopathy characterized by microangiopathic hemolytic anemia, thrombocytopenia, and organ injury. TTP pathophysiology is based on a severe ADAMTS13 deficiency, and is a medical emergency with fatal outcome if appropriate treatment is not initiated promptly. Areas covered Authors will review the best options currently available to minimize mortality, prevent relapses, and obtain the best clinical response in patients with immune TTP (iTTP). Available bibliography about iTTP treatment has been searched in Library's MEDLINE/PubMed database from January 1990 until April 2021. Expert opinion The general…
Thrombotic Thrombocytopenic Purpura: Pathophysiology, Diagnosis, and Management
2021
Thrombotic thrombocytopenic purpura (TTP) is a rare thrombotic microangiopathy characterized by microangiopathic hemolytic anemia, severe thrombocytopenia, and ischemic end organ injury due to microvascular platelet-rich thrombi. TTP results from a severe deficiency of the specific von Willebrand factor (VWF)-cleaving protease, ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type 1 repeats, member 13). ADAMTS13 deficiency is most commonly acquired due to anti-ADAMTS13 autoantibodies. It can also be inherited in the congenital form as a result of biallelic mutations in the ADAMTS13 gene. In adults, the condition is most often immune-mediated (iTTP) whereas congenital TTP (cTT…