Comparison of Plasma Exchange with Plasma Infusion in the Treatment of Thrombotic Thrombocytopenic Purpura

Rock, Gail; Shumak, Kenneth H.; Buskard, Noel A.; Blanchette, Victor S.; Kelton, John G.; Nair, Rama C.; Spasoff, R. A.

doi:10.1056/nejm199108083250604

Cited by 1,707 publications

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References 7 publications

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“…3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20 21 22 23 24 25 26 27 28 29 30 31 32 33 34 35 36 37 38 39 40 41 42 43 44 45 46 47 48 49 50 51 52 53 54 55 56 57 58 59 60 61 62 63 64 65 Introduction Treatment with plasma delivered by either exchange [1] or infusion [2] dramatically changed prognosis of thrombotic thrombocytopenic purpura (TTP) and transformed this deadly disease into a curable form. Twenty-five years ago, a randomized study by the Canadian Apheresis Study Group compared plasma exchange (PE) with plasma infusion and concluded that the former was significantly better than the latter, with 78% of patients surviving six month after entry into the study [3].…”

Section: Discussionmentioning

confidence: 99%

High versus standard dose methylprednisolone in the acute phase of idiopathic thrombotic thrombocytopenic purpura: a randomized study

et al. 2009

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Order of Authors: Carlo L Balduini; Luigi Gugliotta; Mario Luppi; Luca Laurenti; Catherine Klersy; Carla Pieresca; Gerlando Quintini; Francesco Iuliano; Rossana Re; Pierangelo Spedini; Nicola Vianelli; Alfonso Zaccaria; Enrico M Pogliani; Roberto Musso; Enrico Bobbio Pallavicini; Giovanni Quarta; Piero Galieni; Alberto Fragasso; Gianluca Casella; Patrizia Noris; Edoardo Ascari Abstract: Therapeutic plasma exchange (PE) is the accepted therapy for thrombotic thrombocytopenic purpura (TTP). Because not all patients obtain remission, other treatment modalities have been used in addition to PE, but no randomized clinical trial evaluated their efficacy. The aim of this multicentric study was to compare the effectiveness of standard-versus high-dose methylprednisolone as an adjunctive treatment to PE in the acute phase of TTP. Sixty patients with idiopathic TTP were randomized to receive standard-dose (1 mg/kg/die i.v.) or high-dose methylprednisolone (10 mg/kg/die for three days, thereafter 2.5 mg/kg/die) in addition to PE. Both dosages of steroids were well tolerated. At the end of induction therapy (day 23), the percentage of patients failing to obtain complete remission was significantly higher in the standard-dose (16/30) than in the high-dose group (7/30). Also the number of cases without a good response at day 9 and the number of deaths were higher in the standard-dose arm, but the differences did not reach the statistical significance. Results of present study indicate that the association of PE with high-dose instead of standard-dose steroids reduces the percentage of TTP patients that fail to achieve complete remission.Response to Reviewers: Reviewer #1: The authors have proved that the association of plasma exchanged with high-dose methylprednisolone instead of standard-dose reduces the percentage of TTP patients that failed to achieve complete remission. This observation has important clinical implication. I have no major remarksMinor remarks: Activity of ADAMTS 13 and autoantibodies against ADAMTS 13 were not tested. In my opinion this paper can be published in Annals Of Hematology in the present form.Reviewer #2: General:In this randomized clinical trial Balduini and colleagues compared low-dose vs. high dose steroids as an adjunctive treatment to plasma exchange with fresh frozen plasma / cryosupernatant in 60 patients suffering from acute TTP. In the low-dose arm more patients (13/30 vs. 7/30, p= 0.17) failed to achieve a good response at day 9 (primary endpoint of the study) and also more patients failed to achieve complete remission (16/30 vs. 7/30; p= 0.03). Also the number of death (4 vs. 1) was higher, this was however not statistically significant. The authors conclude that plasma exchange in combination with high dose steroids is superior to low-dose steroids. Over the years the Italian TTP study group has been able to conduct several randomized clinical trials in the setting of acute TTP addressing open questions in the field where many other groups have failed to do likewise due to lack of sui...

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Section: Discussionmentioning

confidence: 99%

High versus standard dose methylprednisolone in the acute phase of idiopathic thrombotic thrombocytopenic purpura: a randomized study

et al. 2009

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“…Approximately 80% of patients who are treated aggressively with exchange plasmapheresis survive the initial episode of TTP [2]. Death is frequently due to delay in diagnosis and treatment or ineffectiveness of plasma exchange.…”

Section: Managementmentioning

confidence: 99%

“…Plasma therapy, whose effectiveness in the treatment of TTP was discovered through astute clinical observations and has been validated in randomized control investigation [2], is technically demanding, frequently complicated with transfusion-related complications, and quite costly. When plasma exchange does not lead to remission, clinicians often resort to immunosuppressive drugs or splenectomy, although the effectiveness of these modalities remains unclear.…”

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confidence: 99%

Thrombotic Thrombocytopenic Purpura: A Thrombotic Disorder Caused by ADAMTS13 Deficiency

Tsai

2007

Hematology/Oncology Clinics of North America

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“…The coexistence of SLE and TTP, although rare, has been reported to be more common in children with SLE than in adults [4.]The diagnostic criteria for TTP have changed significantly from the original pentad of thrombocytopenia, microangiopathic hemolytic anemia, neurological abnormalities, renal abnormalities, and fever, [5] to the dyad of thrombocytopenia and microangiopathic hemolytic anemia [6]. The coexistence of 5 symptoms, typical of TTP and often described in textbooks, is not frequent in clinical practice.…”

Section: Discussionmentioning

confidence: 99%

Cryosupernatant and Immunosuppression as Effective Alternative Therapies for TTP in Three Pediatric SLE Patients

Palaniappan

Lionel

Agarwal

et al. 2014

Indian J Hematol Blood Transfus

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Thrombotic thrombocytopenic purpura (TTP) and systemic lupus erythematosus (SLE) very rarely present simultaneously and pose a diagnostic and therapeutic dilemma to the physician. Prompt diagnosis and management with plasma exchange and immunosuppression is life-saving. To describe the effectiveness of cryosupernatant and steroids in pediatric SLE with TTP. We describe three children aged 12-14 years with SLE who were diagnosed with TTP based on fever, CNS manifestations, ANA, anti-dsDNA, anti-sm positivity, hypocomplementemia, and microangiopathic anemia with thrombocytopenia.

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Comparison of Plasma Exchange with Plasma Infusion in the Treatment of Thrombotic Thrombocytopenic Purpura

Abstract: Plasma exchange is more effective than plasma infusion in the treatment of thrombotic thrombocytopenic purpura.

Cited by 1,707 publications

References 7 publications

High versus standard dose methylprednisolone in the acute phase of idiopathic thrombotic thrombocytopenic purpura: a randomized study

High versus standard dose methylprednisolone in the acute phase of idiopathic thrombotic thrombocytopenic purpura: a randomized study

Thrombotic Thrombocytopenic Purpura: A Thrombotic Disorder Caused by ADAMTS13 Deficiency

Cryosupernatant and Immunosuppression as Effective Alternative Therapies for TTP in Three Pediatric SLE Patients

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