Treating TTP/HUS with plasma exchange: a single centre’s 25‐year experience

Forzley, Brian; Sontrop, Jessica M.; Macnab, Jennifer; Chen, Salina; Clark, William F.

doi:10.1111/j.1365-2141.2008.07317.x

Cited by 24 publications

(27 citation statements)

References 30 publications

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“…The nearly 50% distribution of primary and secondary causes mirrors an earlier study from our center as well as a larger study from a Japanese PE center with over 435 900 patients [7,11] . Patients with primary TTP had a higher refractory and relapse rate than those with secondary TTP or HUS, but were more likely to achieve remission and survive.…”

Section: Discussionsupporting

confidence: 58%

Primary and Secondary Thrombotic Microangiopathy Referred to a Single Plasma Exchange Center for Suspected Thrombotic Thrombocytopenic Purpura: 2000-2011

Dahlan

Sontrop

et al. 2015

Am J Nephrol

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Background: Patients who present with unexplained thrombocytopenia, that is, hemolytic anemia with end-organ dysfunction with a normal coagulation profile are suspected to have thrombotic thrombocytopenic purpura (TTP) and are usually referred to a plasma exchange (PE) center for immediate treatment to prevent mortality. Here, we describe the distribution and outcomes of patients with suspected TTP referred to a single center for PE therapy. Methods: In this retrospective cohort study, we reviewed the data of all consecutive patients who were treated with PE for suspected TTP at our center between January 2000 and December 2011 (Canada). Patients were followed for a median of 73 months. Results: Of 137 patients, 70 (51%) were determined to have primary (idiopathic) TTP and 67 (49%) secondary TTP or hemolytic uremic syndrome (HUS). Patients with primary TTP were twice as likely to be refractory than those with secondary TTP or HUS: 27 vs. 12%; p = 0.03. Patients with primary TTP were more likely to experience remission (61/70 (87%) vs. 45/67 (67%); p = 0.01); however, the relapse rate was higher in patients with primary versus secondary TTP-HUS: 11 vs. 1.5%, respectively (p = 0.03). The overall mortality rate was 23% (13 vs. 33% in those with primary vs. secondary TTP-HUS; p = 0.007). After excluding deaths from scleroderma renal crisis (100% mortality), malignancy (75% mortality) and stem cell transplant (50% mortality), the survival rate for secondary TTP was 85%. Conclusions: In contrast to patients with secondary TTP or HUS, those with primary TTP have a higher refractory and relapse rate, but are also more likely to achieve remission and survive.

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Section: Discussionsupporting

confidence: 58%

Primary and Secondary Thrombotic Microangiopathy Referred to a Single Plasma Exchange Center for Suspected Thrombotic Thrombocytopenic Purpura: 2000-2011

Dahlan

Sontrop

et al. 2015

Am J Nephrol

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“…Although it is a common belief that relapses of TTP present with less severe clinical manifestations [7,8,11], there are few studies on which to support such a conviction.…”

Section: Introductionmentioning

confidence: 99%

“…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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“…Plasma therapy was held and regular laboratory monitoring revealed a continuing good response initially, but return of her headache and recurrence of thrombocytopenia with an increase in LDH signaled a relapse of her TTP. Because this occurred within 30 days of her remission, she was labeled as having a re-exacerbation or refractory TTP (23,24).…”

Section: Discussionmentioning

confidence: 99%

Attending Rounds

Clark

Hildebrand

2012

Clinical Journal of the American Society of Nephrology

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SummaryThe classification of thrombotic microangiopathy has evolved and expanded due to treatment and advances in understanding of the diseases associated with this clinical presentation. The three clinical forms of thrombotic microangiopathy-thrombotic thrombocytopenic purpura (TTP), hemolytic uremic syndrome (HUS), and disseminated intravascular coagulation-encompass a wide range of disorders that can be classified as either primary (idiopathic) or secondary to another identifiable disease or clinical context. Identification of an inhibitor to a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 (ADAMTS13) in the idiopathic and acute forms of TTP, recognition of the absence of ADAMTS13 inhibition in diarrheal HUS, identification of complement abnormalities in atypical HUS, and a better understanding of the role of plasma therapy, rituximab, and eculizumab therapy have all had a major effect on current understanding of the thrombotic microangiopathies. In this Attending Rounds, a patient with a thrombotic microangiopathy is presented, along with discussion highlighting the difficulty of differentiating TTP from HUS and disseminated intravascular coagulation, the need for a prompt diagnosis, and the role for plasma therapy in appropriately selected patients. The discussion attempts to provide a simple clinical approach to the diagnosis, treatment options, and future course of adults and children suffering from a thrombotic microangiopathy.

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Treating TTP/HUS with plasma exchange: a single centre’s 25‐year experience

Cited by 24 publications

References 30 publications

Primary and Secondary Thrombotic Microangiopathy Referred to a Single Plasma Exchange Center for Suspected Thrombotic Thrombocytopenic Purpura: 2000-2011

Primary and Secondary Thrombotic Microangiopathy Referred to a Single Plasma Exchange Center for Suspected Thrombotic Thrombocytopenic Purpura: 2000-2011

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

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