The pathophysiology and management of thrombotic thrombocytopenic purpura

Ruggenenti, Piero; Remuzzi, Giuseppe

doi:10.1111/j.1600-0609.1996.tb01930.x

Cited by 111 publications

(17 citation statements)

References 118 publications

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“…The reason for the observed increased CSA excretion in HUS patients is unknown. In its typical presentation, HUS manifests as an acute disease and 80% to 90% of cases recover without sequelae, either spontaneously (as in most cases of childhood HUS) or after plasma infusion or exchange (as in adult or severe forms of HUS) [28,33]. In sample 10 b lower FSA and CSA concentrations are found in comparison with urine specimen 10 a , indicating that the patients condition is improved.…”

Section: Discussionmentioning

confidence: 92%

Quantification of free and total sialic acid excretion by LC–MS/MS

Ham

Prinsen

Huijmans

et al. 2007

Journal of Chromatography B

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Section: Discussionmentioning

confidence: 92%

Quantification of free and total sialic acid excretion by LC–MS/MS

Ham

Prinsen

Huijmans

et al. 2007

Journal of Chromatography B

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“…Single agent response rates for the antiplatelet agents aspirin and dipyridamole approximate 10%,49,180 a result indistinguishable from the natural history of TTP. Antiplatelet agents have not been convincingly shown to increase the response to plasma exchange45,51,181 and may promote bleeding in the setting of acute thrombocytopenia and invasive procedures 51,182.…”

Section: Therapeutic Modalitiesmentioning

confidence: 87%

Thrombotic Microangiopathy in Haematopoietic Cell Transplantation:an Update

Stavrou

Lazarus

2010

Mediterr J Hematol Infect Dis

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Allogeneic hematopoietic cell transplantation (HCT) represents a vital procedure for patients with various hematologic conditions. Despite advances in the field, HCT carries significant morbidity and mortality. A rare but potentially devastating complication is transplantation-associated thrombotic microangiopathy (TA-TMA). In contrast to idiopathic TTP, whose etiology is attributed to deficient activity of ADAMTS13, (a member of the A Disintegrin And Metalloprotease with Thrombospondin 1 repeats family of metalloproteases), patients with TA-TMA have > 5% ADAMTS13 activity. Pathophysiologic mechanisms associated with TA-TMA, include loss of endothelial cell integrity induced by intensive conditioning regimens, immunosuppressive therapy, irradiation, infections and graft-versus-host (GVHD) disease. The reported incidence of TA-TMA ranges from 0.5% to 75%, reflecting the difficulty of accurate diagnosis in these patients. Two different groups have proposed consensus definitions for TA-TMA, yet they fail to distinguish the primary syndrome from secondary causes such as infections or medication exposure. Despite treatment, mortality rate in TA-TMA ranges between 60% to 90%. The treatment strategies for TA-TMA remain challenging. Calcineurin inhibitors should be discontinued and replaced with alternative immunosuppressive agents. Daclizumab, a humanized monoclonal anti-CD25 antibody, has shown promising results in the treatment of TA-TMA. Rituximab or the addition of defibrotide, have been reported to induce remission in this patient population. In general, plasma exchange is not recommended.

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“…plasma exchange or infusion) aimed at stopping the microangiopathic process and TTP should always be tried. However, this approach is seldom effective in secondary forms, where the outcome mainly depends on the prognosis of the underlying condition [12] . Therefore, specific therapy should be started as soon as the diagnosis of the predisposing condition has been established in order to effect disease recovery and minimize the risk of sequelae.…”

Section: Discussionmentioning

confidence: 99%

Thrombotic Thrombocytopenic Purpura in a Patient with Brucella Infection Is Highly Responsive to Combined Plasma Infusion and Antimicrobial Therapy

Erdem¹,

Kıkı²,

Gündoğdu³

et al. 2007

Med Princ Pract

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Objective: To report a case of brucella infection presenting with thrombotic thrombocytopenic purpura (TTP) that responded well to plasma and antimicrobial treatment infusion. Case Presentation and Intervention: A 51-year-old man with moderate confusion, depressed mood and dysarthria was admitted. He was chronically ill, with fever (38.5°C), anemia, jaundice and petechial-purpuric skin lesions. Neurological examination revealed diminished consciousness with a Glasgow coma scale score of 7 and +1 neck rigidity. The hemoglobin and platelet counts were decreased and reticulocyte index, erythrocyte sedimentation rate, as well as serum lactate dehydrogenase and renal dysfunction were elevated. TTP was a possible diagnosis and the patient responded well to plasma infusion and antimicrobial treatment. Conclusion: This report shows that therapy of underlying infection together with plasma infusion may be a successful treatment option for brucellosis-induced TTP.

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The pathophysiology and management of thrombotic thrombocytopenic purpura

Cited by 111 publications

References 118 publications

Quantification of free and total sialic acid excretion by LC–MS/MS

Quantification of free and total sialic acid excretion by LC–MS/MS

Thrombotic Microangiopathy in Haematopoietic Cell Transplantation:an Update

Thrombotic Thrombocytopenic Purpura in a Patient with Brucella Infection Is Highly Responsive to Combined Plasma Infusion and Antimicrobial Therapy

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