Late Relapses in Patients Successfully Treated for Thrombotic Thrombocytopenic Purpura

Shumak, Kenneth H.; Rock, Gail; Nair, Rama C.

doi:10.7326/0003-4819-122-8-199504150-00002

Cited by 155 publications

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“…[26][27][28][29][30] In the South East England TTP registry, Ͼ 50% of the recorded episodes were relapses. 14 Relapse in TTP BLOOD, 18 AUGUST 2011 ⅐ VOLUME 118, NUMBER 7 For personal use only.…”

Section: Discussionmentioning

confidence: 99%

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

362

375

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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%

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

362

375

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“…Moreover, differences between authors in the definition of relapse and the inclusion of patients with haemolytic uremic syndrome or secondary TTP in some studies further compound drawing the specific features of relapsed as compared with de novo idiopathic TTP [7,8,20,21,23].…”

Section: Introductionmentioning

confidence: 99%

Newly diagnosed versus relapsed idiopathic thrombotic thrombocytopenic purpura: a comparison of presenting clinical characteristics and response to treatment

et al. 2009

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et al.. Newly diagnosed versus relapsed idiopathic thrombotic thrombocytopenic purpura: a comparison of presenting clinical characteristics and response to treatment. Annals of Hematology, Springer Verlag, 2009, 88 (10), pp.973-978. 10.1007/s00277-009-0707-9 . hal-00535035 ORIGINAL ARTICLENewly diagnosed versus relapsed idiopathic thrombotic thrombocytopenic purpura: a comparison of presenting clinical characteristics and response to treatment Abstract The remission rate with plasma exchange (PE) in thrombotic thrombocytopenic purpura (TTP) exceeds 80%, but the disease relapses in up to 20-30% of the cases. Clinical characteristics and response to treatment of relapsed TTP are not well defined. The objective of the present study was to compare the clinical and biological characteristics at presentation and the response to treatment between de novo and relapsed TTP. For such purpose, a total of 102 episodes of idiopathic TTP (70 de novo and 32 relapses) included in a recent multicentric prospective cohort study were analysed. All patients were homogeneously treated with daily PE and costicosteroids. In comparison with de novo TTP, episodes of relapsed TTP showed a higher Hb level (median, 122 g/l versus 91 g/l, p < 0.001) and lower serum lactate dehydrogenase (2.2-versus 4.5-fold above the upper limit of normality, p < 0.001). Neurological symptoms and fever were less frequently observed in patients with relapsed TTP than in patients with de novo TTP. Patients with relapsed TTP needed fewer PE sessions Ann Hematol (2009) 88:973-978 DOI 10.1007 Alberto Alvarez-Larrán and Julio del Río-Garma contributed equally to this article. (five versus ten, p = 0.02) and a smaller volume of plasma (221 ml/kg versus 468 ml/kg, p = 0.004) to achieve remission than those with de novo TTP. There was no significant difference in the rate of recrudescence under treatment, the need of complementary treatments or the frequency of refractoriness to PE therapy. In conclusion, relapsed TTP has a milder clinical profile and responds more easily to PE than de novo TTP.

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“…In Shumak et al [6] study, 63 patients who recovered from an acute episode of TTP were analyzed to determine the incidence and timing of relapses during clinical follow up ranged from 3 to 10 years. One or more episodes of relapse were documented in 17/63 (27%) patients, occurring 7 months to 8 years after the original episode.…”

Section: Discussionmentioning

confidence: 99%

“…15/20 female had uncomplicated pregnancies/deliveries before initial episode. Ten of them (n 0 1,4,5,6,8,12,13,19,20,21) had 2 pregnancies/deliveries while five of them (n 0 2,9,15,17,22) had 1 pregnancy/delivery before first episode. In one patient (n 0 16) initial episode was associated with 38 gestation week of her first pregnancy.…”

Section: Uncomplicated Pregnancy/delivery Secondary To Previous Episomentioning

confidence: 99%

“…In majority of cases, relapses are associated with persistent or recurrent severe protease deficiency which is almost totally restricted to patients who have idiopathic TTP [5]. Recurrent episodes of disease occur in up to 50% of patients who survive their initial episode of TTP [3,[5][6][7]. Relapses usually develop during the first year after the initial episode and most patients will have only one relapse [2].…”

Section: Introductionmentioning

confidence: 99%

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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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Late Relapses in Patients Successfully Treated for Thrombotic Thrombocytopenic Purpura

Abstract: More than one third of patients who survive an acute episode of thrombotic thrombocytopenic purpura will have at least one relapse during the following 10 years.

Cited by 155 publications

References 0 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

Newly diagnosed versus relapsed idiopathic thrombotic thrombocytopenic purpura: a comparison of presenting clinical characteristics and response to treatment

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

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