scholarly journals How I treat patients with thrombotic thrombocytopenic purpura–hemolytic uremic syndrome

Blood ◽  
2000 ◽  
Vol 96 (4) ◽  
pp. 1223-1229 ◽  
Author(s):  
James N. George
Keyword(s):  
Renal Failure ◽  
Hemolytic Uremic Syndrome ◽  
Plasma Exchange ◽  
Thrombotic Thrombocytopenic Purpura ◽  
Survival Rates ◽  
Thrombocytopenic Purpura ◽  
Long Term Follow Up ◽  
Uremic Syndrome

Thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS) are, in adults, clinically and pathologically indistinguishable except for the severity of renal failure. They are best described as a single disorder, TTP-HUS, because the diagnostic evaluation and initial management are the same. Treatment with plasma exchange, available for more than 20 years, has dramatically altered the course of disease in adults with TTP-HUS. Plasma exchange has improved survival rates from 10% to between 75% and 92%, creating urgency for the initiation of treatment. This has resulted in decreased stringency of diagnostic criteria, which in turn has resulted in a broader spectrum of disorders for which the diagnosis of TTP-HUS is considered. Long-term follow-up has revealed increasing frequencies of relapse and of chronic renal failure. Although the increased survival rate is dramatic and recent advances in understanding the pathogenesis of these syndromes are remarkable, clinical decisions remain empirical. Therefore, the management decisions for patients with suspected TTP-HUS rely on individual experience and opinion, resulting in many different practice patterns. Multipractice clinical trials are required to define optimal management.

scholarly journals How I treat patients with thrombotic thrombocytopenic purpura–hemolytic uremic syndrome

Blood ◽  
2000 ◽  
Vol 96 (4) ◽  
pp. 1223-1229 ◽  
Author(s):  
James N. George
Keyword(s):  
Renal Failure ◽  
Hemolytic Uremic Syndrome ◽  
Plasma Exchange ◽  
Thrombotic Thrombocytopenic Purpura ◽  
Survival Rates ◽  
Thrombocytopenic Purpura ◽  
Long Term Follow Up ◽  
Uremic Syndrome

Abstract Thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS) are, in adults, clinically and pathologically indistinguishable except for the severity of renal failure. They are best described as a single disorder, TTP-HUS, because the diagnostic evaluation and initial management are the same. Treatment with plasma exchange, available for more than 20 years, has dramatically altered the course of disease in adults with TTP-HUS. Plasma exchange has improved survival rates from 10% to between 75% and 92%, creating urgency for the initiation of treatment. This has resulted in decreased stringency of diagnostic criteria, which in turn has resulted in a broader spectrum of disorders for which the diagnosis of TTP-HUS is considered. Long-term follow-up has revealed increasing frequencies of relapse and of chronic renal failure. Although the increased survival rate is dramatic and recent advances in understanding the pathogenesis of these syndromes are remarkable, clinical decisions remain empirical. Therefore, the management decisions for patients with suspected TTP-HUS rely on individual experience and opinion, resulting in many different practice patterns. Multipractice clinical trials are required to define optimal management.

Long-term Efficacy of Rituximab Treatment in Thrombotic Thrombocytopenic Purpura

2011 ◽  
Vol 07 (02) ◽  
pp. 143
Author(s):  
Jens M Chemnitz ◽  
Michael Hallek ◽  
Christof Scheid ◽  
◽  
◽  
...  
Keyword(s):  
Plasma Exchange ◽  
Thrombotic Thrombocytopenic Purpura ◽  
Case Reports ◽  
Therapeutic Option ◽  
Case Series ◽  
Current Data ◽  
Thrombocytopenic Purpura ◽  
Long Term Follow Up

The use of therapeutic plasma exchange has reduced mortality rates in thrombotic thrombocytopenic purpura (TTP) from 90 to 10–20%. However, TTP is a potentially lethal disorder, and management of patients with TTP refractory to plasma exchange or frequently recurrent disease is difficult. In those cases, rituximab might be a therapeutic option, although current data are based primarily on case reports and smaller case series. While initial response rates to rituximab are reported to be high, long-term follow-up data of patients treated with rituximab are rare; however, it is important to estimate the safety and benefit of this treatment. In this article we focus on current experience with rituximab in the treatment of TTP, including recent results with long-term follow-up.

scholarly journals Long-term follow-up of children with typical hemolytic uremic syndrome

Medicina ◽  
2015 ◽  
Vol 51 (3) ◽  
pp. 146-151
Author(s):  
Birutė Pundzienė ◽  
Diana Dobilienė ◽  
Rimantė Čerkauskienė ◽  
Renata Mitkienė ◽  
Aina Medzevičienė ◽  
...  
Keyword(s):  
Hemolytic Uremic Syndrome ◽  
Long Term Follow Up ◽  
Uremic Syndrome

Long-term follow-up of Argentinean patients with hemolytic uremic syndrome who had not undergone dialysis

2007 ◽  
Vol 22 (9) ◽  
pp. 1343-1347 ◽  
Author(s):  
Carlos J. Cobeñas ◽  
Laura F. Alconcher ◽  
Ana P. Spizzirri ◽  
Ricardo C. Rahman
Keyword(s):  
Hemolytic Uremic Syndrome ◽  
Long Term Follow Up ◽  
Uremic Syndrome

scholarly journals Open ADAMTS13, induced by antibodies, is a biomarker for subclinical immune-mediated thrombotic thrombocytopenic purpura

Blood ◽  
2020 ◽  
Author(s):  
Elien Roose ◽  
An-Sofie Schelpe ◽  
Edwige Tellier ◽  
György Sinkovits ◽  
Bérangère S Joly ◽  
...  
Keyword(s):  
Thrombotic Thrombocytopenic Purpura ◽  
Adamts13 Activity ◽  
Thrombocytopenic Purpura ◽  
Follow Up Study ◽  
Open Conformation ◽  
Immune Mediated ◽  
Long Term Follow Up ◽  
Donor Plasma

Recently, we showed that during the acute phase of immune-mediated thrombotic thrombocytopenic purpura (iTTP), ADAMTS13 circulates in an open conformation. Although the cause of this conformational change in acute iTTP remains elusive, ADAMTS13 is mainly closed in iTTP patients (i) in remission with an ADAMTS13 activity >50% and undetectable anti-ADAMTS13 autoantibodies, and (ii) after rituximab treatment, suggesting a role for anti-ADAMTS13 autoantibodies. Therefore, IgGs from 18 acute iTTP patients were purified and added to closed ADAMTS13 in healthy donor plasma. This resulted in open ADAMTS13 in 14/18 (78%) samples, proving that indeed anti-ADAMTS13 autoantibodies can induce an open ADAMTS13 conformation. To further elucidate the conformation of ADAMTS13 in iTTP patients, we studied a novel iTTP patient cohort (n=197) that also included plasma samples of iTTP patients in remission where ADAMTS13 activity was <50%. The open ADAMTS13 conformation was not only found during acute iTTP but also in patients in remission with an ADAMTS13 activity <50% and in half of the patients with an ADAMTS13 activity >50%, although free anti-ADAMTS13 autoantibodies were not always detected. Thus open ADAMTS13 is not only a hallmark of acute iTTP, but also a novel biomarker to detect subclinical iTTP in patients in remission. Finally, a long term follow-up study in one iTTP patient showed that the open conformation precedes a severe drop in ADAMTS13 activity. In conclusion, we have shown that anti-ADAMTS13 autoantibodies from iTTP patients induce an open ADAMTS13 conformation. Most importantly, an open ADAMTS13 conformation is a biomarker for subclinical iTTP and could become an important tool in TTP management.

scholarly journals Thrombotic Thrombocytopenic Purpura Associated with Pneumococcal Sepsis

1993 ◽  
Vol 4 (3) ◽  
pp. 145-147 ◽  
Author(s):  
Jeffrey R Schriber ◽  
John J Freedman ◽  
Joseph M Brandwein
Keyword(s):  
Renal Failure ◽  
Streptococcus Pneumoniae ◽  
Hemolytic Uremic Syndrome ◽  
Thrombotic Thrombocytopenic Purpura ◽  
Antiplatelet Agents ◽  
Microangiopathic Hemolytic Anemia ◽  
Thrombocytopenic Purpura ◽  
Pneumococcal Sepsis ◽  
Oliguric Renal Failure ◽  
Uremic Syndrome

The first documented case of thrombotic thrombocytopenic purpura (TTP) associated with pneumococcal septicemia is reported. This association has been previously demonstrated with hemolytic uremic syndrome. The patient presented with recurrent seizures, oliguric renal failure, fever, thrombocytopenia and microangiopathic hemolytic anemia; coagulation studies were normal. Blood and sputum cultures were positive forStreptococcus pneumoniae.The patient responded to therapy with plasmapheresis and antiplatelet agents as well as antibiotics. Coincident infection should be searched for in all cases of TTP.

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