Guidelines on the diagnosis and management of the thrombotic microangiopathic haemolytic anaemias

Allford, Sarah L.; Hunt, Beverley J.; Rose, Peter; Machin, Samuel J.

doi:10.1046/j.1365-2141.2003.04049.x

Cited by 347 publications

(261 citation statements)

References 179 publications

(199 reference statements)

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“…The criteria used as indications for the use of additional therapies were failure to achieve a normal platelet count at 1 week and/or deterioration in clinical symptoms. 2 Fifty percent of these patients subsequently relapsed and 2 patients died. In those historic control patients treated with PEX with or without steroids (and no additional immunosuppression), 50% also relapsed.…”

Section: Discussionmentioning

confidence: 99%

“…Historical control patients similarly followed the British Committee for Standards in Haematology guidelines. 2 Cryosupernatant and Octaplas were also available before January 2006.…”

Section: Study Design and Methodologymentioning

confidence: 99%

“…The mainstay of treatment remains plasma exchange (PEX) and steroids. 2 However, patients with acquired idiopathic autoimmune disease are more likely to take longer to achieve a normal platelet count and require more plasma therapy, 3 with a relapse rate of 30%-50%. 4,5 In such refractory/relapsing TTP patients, further immunosuppressive therapy may be required, such as Ciclosporin, cyclophosphamide, or vincristine.…”

Section: Introductionmentioning

confidence: 99%

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A phase 2 study of the safety and efficacy of rituximab with plasma exchange in acute acquired thrombotic thrombocytopenic purpura

Scully

McDonald

Cavenagh

et al. 2011

Blood

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368

388

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The safety and efficacy of weekly rituximab 375 mg/m 2 (؋4), given within 3 days of acute TTP admission, with standard therapy (PEX and steroids) was evaluated. Clinical outcomes were compared to historical controls (n ‫؍‬ 40) who had not received rituximab. Within the trial group, 15 of 40 required ICU admission and 15% of all cases with the highest troponin T levels on admission were ventilated. Before the second rituximab infusion, 68% of cases had a platelet count > 50 ؋ 10 9 /L and 38% > 150 ؋ 10 9 /L. Fewer PEX were required in whites compared to nonwhite in the rituximab group (mean 14 vs 21, P ‫؍‬ .0095). Inpatient stay was reduced by 7 days in the non-ICU trial cases compared to historical controls (P ‫؍‬ .04), especially in whites, with a mean reduction of 7 days (P ‫؍‬ .05). Ten percent of trial cases relapsed, median, 27 months (17-31 months), compared to 57% in historical controls, median 18 months (3-60 months; P ‫؍‬ .0011).There were no excess infections or serious adverse events with rituximab. In conclusion, rituximab appears a safe and effective therapy. Inpatient stay and relapse are significantly reduced in the rituximab cohort. Rituximab should be considered in conjunction with standard therapy on acute presentation of TTP. This study was registered at www.clinicaltrials.gov as NCT009-3713. (Blood. 2011;118(7): 1746-1753)

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Section: Discussionmentioning

confidence: 99%

“…Historical control patients similarly followed the British Committee for Standards in Haematology guidelines. 2 Cryosupernatant and Octaplas were also available before January 2006.…”

Section: Study Design and Methodologymentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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A phase 2 study of the safety and efficacy of rituximab with plasma exchange in acute acquired thrombotic thrombocytopenic purpura

Scully

McDonald

Cavenagh

et al. 2011

Blood

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368

388

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“…Refractory patients are often treated with cryoprecipitate-depleted plasma, vincristine, splenectomy, cyclophosphamide, azathioprine, splenectomy, high dose intravenous immunoglobulins or prostacyclin analogues, mostly in anecdotal case reports or with questionable responses [90]. More recently, rituximab, a chimeric monoclonal anti-CD20, has been used with presumed benefits in patients with protracted TTP.…”

Section: Managementmentioning

confidence: 99%

Thrombotic Thrombocytopenic Purpura: A Thrombotic Disorder Caused by ADAMTS13 Deficiency

Tsai

2007

Hematology/Oncology Clinics of North America

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“…Complete remission was defined as no plasma exchange treatment at least 30 days after laboratory and clinical normalization: normal platelet count for 2 consecutive days, recovery of LDH and haptoglobin to normal, normalization of organs function with a rising hemoglobin [4,11,12,17].…”

Section: Complete Remissionmentioning

confidence: 99%

Recurrent Disease in 25 Patients with Acquired Thrombotic Thrombocytopenic Purpura and Hemolytic Uremic Syndrome during 6.5 Years Follow-Up

Vũcelić¹

2017

HTIJ

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Thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS) are manifestations of excessive microvascular platelet aggregation and present dangerous thrombotic microangiopathies (TMA). The clinical presentation of TTP/HUS is variable in terms of type and severity of clinical symptoms. Before the introduction of plasma exchange (PEX) for the treatment of acute TTP episode mortality reached 90%, but decreased to 8-30% following the adoption of PEX. Acute idiopathic TTP is the most common type of TTP seen in clinical practice. The clinical course of idiopathic TTP is characterized by recurrent disease episodes in up to 50% of cases; with a higher risk of recurrence in those with severe deficiency of the von Willebrand factor cleavingprotease ADAMTS13 during acute episodes. We report on the recurrences among 25 patients with TTP and HUS, in whom we monitored routine laboratory parameters, response to treatment as well as ADAMTS13 activity and anti-ADAMTS13 antibodies in acute phase and remission.

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Guidelines on the diagnosis and management of the thrombotic microangiopathic haemolytic anaemias

Cited by 347 publications

References 179 publications

A phase 2 study of the safety and efficacy of rituximab with plasma exchange in acute acquired thrombotic thrombocytopenic purpura

A phase 2 study of the safety and efficacy of rituximab with plasma exchange in acute acquired thrombotic thrombocytopenic purpura

Thrombotic Thrombocytopenic Purpura: A Thrombotic Disorder Caused by ADAMTS13 Deficiency

Recurrent Disease in 25 Patients with Acquired Thrombotic Thrombocytopenic Purpura and Hemolytic Uremic Syndrome during 6.5 Years Follow-Up

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