Current understanding of severe preeclampsia, pregnancy-associated hemolytic uremic syndrome, thrombotic thrombocytopenic purpura, hemolysis, elevated liver enzymes, and low platelet syndrome, and postpartum acute renal failure: different clinical syndromes or just different names?

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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Postpartum renal failure: a complex case with probable coexistence of hemolysis, elevated liver enzymes, low platelet count, and hemolytic uremic syndrome

Obstetrics and Gynecology ◽

10.1016/s0029-7844(98)00196-3 ◽

1998 ◽

Vol 92 (4) ◽

pp. 698-700 ◽

Cited By ~ 9

Author(s):

K KAHRA

Keyword(s):

Renal Failure ◽

Platelet Count ◽

Hemolytic Uremic Syndrome ◽

Liver Enzymes ◽

Complex Case ◽

Elevated Liver Enzymes ◽

Uremic Syndrome ◽

Low Platelet Count

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

Blood ◽

10.1182/blood.v96.4.1223 ◽

2000 ◽

Vol 96 (4) ◽

pp. 1223-1229 ◽

Cited By ~ 237

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.

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

Blood ◽

10.1182/blood.v96.4.1223.h8001223_1223_1229 ◽

2000 ◽

Vol 96 (4) ◽

pp. 1223-1229 ◽

Cited By ~ 2

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.

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