losartan-potassium and Purpura--Thrombotic-Thrombocytopenic

Large and unusually large von Willebrand factor (vWf) multimers may be responsible for systemic platelet aggregation in thrombotic thrombocytopenic purpura (TTP). This possibility is supported by studies that show deficient vWf-cleaving metalloproteinase and increased platelet-vWf binding during TTP episodes. In acute idiopathic TTP, decreased vWf metalloproteinase is the result of autoantibodies against the enzyme. In familial and acquired hemolytic-uremic syndrome, vWf-cleaving metalloproteinase activity is normal. A deficiency or defect in factor H, which normally dampens the activation of C3 via the alternative complement pathway, has been seen in some patients with familial hemolytic-uremic syndrome. Ticlopidine therapy is an important risk factor for TTP.

Topics: Anti-Bacterial Agents; Child; Diagnosis, Differential; Erythropoietin; Hemolytic-Uremic Syndrome; Humans; Plasma Exchange; Purpura, Thrombotic Thrombocytopenic; Survival Analysis; Ticlopidine

Thrombotic thrombocytopenic purpura (TTP) is a hematologic emergency characterized by microangiopathic hemolytic anemia and thrombocytopenia. Plasma exchange is the standard treatment. Treating TTP without plasma exchange is a challenge. Due to religious beliefs, Jehovah's Witnesses do not accept transfusions of blood products. We report a case of successful treatment of TTP in a Jehovah's Witness using plasma exchange with albumin replacement.

Topics: ADAM Proteins; ADAMTS13 Protein; Adult; Albumins; Dexamethasone; Disease Management; Erythropoietin; Female; Humans; Immunoglobulins, Intravenous; Jehovah's Witnesses; Plasma Exchange; Platelet Count; Purpura, Thrombotic Thrombocytopenic; Recombinant Proteins; Rituximab; Vincristine

In this study, erythropoietin serum levels were serially determined in eight patients with acute renal failure to get a lead on the etiology of anemia in acute renal failure and to address the relationship between erythropoietin synthesis and renal excretory performance. Erythropoietin serum levels rapidly decreased after onset of acute renal failure to values of 12.8 +/- 10.3 mU/ml compared to 16.8 +/- 9.4 mU/ml in healthy controls. After restoration of renal function, erythropoietin levels climbed slowly in six patients (15.2 +/- 5.3 mU/ml), and in relation to prolonged anemia in these patients, a relative deficiency of erythropoietin could be observed. In one patient with thrombotic thrombocytopenic purpura causing acute renal failure, the decline of erythropoietin secretion was not observed, and in a phase of the disease when plasma exchange therapy was interrupted, markedly increased erythropoietin levels, up to 182 mU/ml, were detected despite the renal failure. Focusing on erythropoietin secretion in thrombotic thrombocytopenic purpura, we followed hormone synthesis in two other patients with the same disease, one of whom had mild renal insufficiency and one had normal renal function. High erythropoietin levels of up to 205 mU/ml were found in these patients, similar to the peak levels found in the patient with complete renal failure. Plasmapheresis treatment reduced erythropoietin production in all three patients with thrombotic thrombocytopenic purpura. In summary, our study indicates that in most cases of acute renal failure, erythropoietin synthesis is compromised and may contribute to the development of anemia in renal failure and aggravate the persistence of anemia after restoration of renal function.(ABSTRACT TRUNCATED AT 250 WORDS)

Topics: Acute Kidney Injury; Adult; Creatinine; Erythropoietin; Female; Hematocrit; Humans; Kidney Function Tests; Male; Purpura, Thrombotic Thrombocytopenic