Treatment of acquired Thrombotic Thrombocytopenic Purpura in the U.S. remains heterogeneous: Current and future points of clinical equipoise

Mazepa, Marshall A.; Raval, Jay S.; Brecher, Mark E.; Park, Yara A.

doi:10.1002/jca.21600

Cited by 15 publications

(16 citation statements)

References 17 publications

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“…Rituximab is commonly used to treat mainly refractory and relapsed iTTP patients 10 . In our series, rituximab was administered as first‐line choice only in 14% of patients at presentation, similarly to the main centers in North America (reviewed by Mazepa et al) 6,9 . We also observed a higher incidence of administration of rituximab as second‐line therapy intended to treat refractoriness or exacerbations, reflecting the more aggressive immunosuppressive approach also used by other groups aimed to prevent further relapses 10,23 .…”

Section: Discussionsupporting

confidence: 59%

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Incidence, diagnosis, and outcome of immune‐mediated thrombotic thrombocytopenic purpura: A nationwide survey by the Spanish registry of thrombotic thrombocytopenic purpura

Pascual‐Izquierdo

Río-Garma

Rubia

et al. 2021

J of Clinical Apheresis

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Background Immune‐mediated thrombotic thrombocytopenic purpura (iTTP) is a rare disease characterized by the presence of anti‐ADAMTS13 autoantibodies. Achieving accurate information on incidence and customary disease management is important to provide appropriate diagnostic and therapeutic resources. The aim of this study was to determine the incidence and outcomes of iTTP in Spain. Study design and methods A cross‐sectional survey was carried out among Spanish hospitals, focused on iTTP patients ≥16 years old attended between 2015 and 2017, and those at follow‐up before that interval. Incidence, prevalence, mortality, refractoriness, exacerbations, treatment complications, relapses, and sequelae were estimated. Results Forty‐two hospitals covering roughly 20 million inhabitants answered the survey and reported 203 episodes (138 newly‐diagnosed and 65 relapses), of which 193 (95.1%) were treated. Incidence was 2.67 (95% CI 1.90‐3.45) patients per million inhabitants per year and prevalence 21.44 (95% CI% 19.10‐23.73) patients per million inhabitants. At diagnosis, ADAMTS13 activity and anti‐ADAMTS13 autoantibody were measured in 97% and 84.3% of reported episodes, respectively. Fifteen patients (7.4%) died as a direct consequence of iTTP, 6 of them before receiving any iTTP‐specific treatment. Thirty‐one (16.1%) of the 193 treated episodes were refractory to plasma exchange and corticosteroids, and 51 (26.4%) suffered at least one exacerbation. Conclusion iTTP incidence and prevalence were somewhat higher than those documented in neighboring countries. Together with data on treatments and outcomes, this information will allow us to better estimate what is needed to improve diagnosis and prognosis of iTTP patients in Spain.

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Section: Discussionsupporting

confidence: 59%

“…Disease management varies according to centers and countries 7‐10 . In Spain, the public health system offers universal coverage to 99.1% of the population, 11 and hematology departments routinely offer an integrated diagnostic and therapeutic approach to patients with any type of hematological disorder.…”

Section: Introductionmentioning

confidence: 99%

Incidence, diagnosis, and outcome of immune‐mediated thrombotic thrombocytopenic purpura: A nationwide survey by the Spanish registry of thrombotic thrombocytopenic purpura

Pascual‐Izquierdo

Río-Garma

Rubia

et al. 2021

J of Clinical Apheresis

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“…However, this proposed superiority in efficacy was not demonstrated in a randomized trial of 27 patients conducted by the North American TTP group (15). Furthermore, as cryosupernatant is not readily available in most US institutions, including ours, our clinical experience is limited as compared to our Canadian colleagues who tend to favor cryosupernatant over FFP in the management of TTP (16,17).…”

Section: Discussionmentioning

confidence: 90%

Successful Management of Thrombotic Thrombocytopenic Purpura in a Jehovah’s Witness: An Individualized Approach With Joint Decision-Making

Lim

Greenberg

2019

Journal of Patient Experience

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The management of thrombotic thrombocytopenic purpura (TTP) presents a unique challenge in individuals who are unable to accept plasma due to religious beliefs, given that therapeutic plasma exchange (TPE) is the standard of care. A 61-year-old Jehovah’s Witness woman presented to our hospital with neurological symptoms and laboratory findings suggestive of TTP. On admission, she refused transfusion of blood products, specifically red blood cells, platelets, and plasma but accepted albumin and intravenous immunoglobulin (IVIG); fractions of plasma. She was started on steroids, IVIG, and TPE with albumin as replacement therapy with minimal improvement. After a detailed discussion with the patient and family, they agreed to accept cryosupernatant. The patient started TPE with cryosupernatant for replacement therapy, which resulted in clinical improvement. This case highlights the importance of an individualized approach with joint decision-making given the significant heterogeneity that exists in Jehovah’s Witnesses’ attitude toward the receipt of blood products.

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“…As the first-line treatment, TPE can replenish ADAMTS13 and block microvascular thrombosis [1–3]. It has been reported that several types of plasma products could be used as the replacement fluid in TPE for patients with TTP [5–9, 16–19]. CP lacking vWF contains an equivalent amount of ADAMTS13 to FFP or its derivative and is considered a better choice than other plasma products, such as FFP, for TPE in patients with TTP [1–3].…”

Section: Discussionmentioning

confidence: 99%

Combination of Fresh Frozen Plasma and Cryosupernatant Plasma for Therapeutic Plasma Exchange in Thrombotic Thrombocytopenic Purpura: A Single Institution Experience

Lin

Fan

Huang

et al. 2019

BioMed Research International

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Purpose. To evaluate the impact of a combination of fresh frozen plasma (FFP) and cryosupernatant plasma (CP) as a replacement fluid in therapeutic plasma exchange (TPE) on early therapeutic response and long-term survival of patients with thrombotic thrombocytopenic purpura (TTP). Materials and Methods. A total of 44 patients with suspected TTP were screened by Bentley and PLASMIC scores. Twenty-seven patients treated with TPE using the FFP and CP combination as the replacement fluid were enrolled and divided into two groups: 11 patients who received TPE with CP-dominant replacement fluid (FFP/CP<1) and 16 patients who received TPE with FFP-dominant replacement fluid (FFP/CP>1). Results. There were no significant differences in the demographic and clinicopathological characteristics between the two groups except for the international normalized ratio (INR). The number of TPE procedures was lower, and time to achieve complete response was shorter in the CP-dominant group than in the FFP-dominant group. There were no significant differences in overall survival between the two groups. Conclusion. The CP-dominant replacement fluid was superior to the FFP-dominant replacement fluid in early response to TPE in patients with TTP, but did not impact the patients’ overall survival.

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Treatment of acquired Thrombotic Thrombocytopenic Purpura in the U.S. remains heterogeneous: Current and future points of clinical equipoise

Abstract: Treatment of acquired TTP in the U.S. remains heterogeneous. Points of clinical equipoise identified were PV exchanged (1.0 vs >1.0), tapering of TPE versus none, and rituximab use.

Cited by 15 publications

References 17 publications

Incidence, diagnosis, and outcome of immune‐mediated thrombotic thrombocytopenic purpura: A nationwide survey by the Spanish registry of thrombotic thrombocytopenic purpura

Incidence, diagnosis, and outcome of immune‐mediated thrombotic thrombocytopenic purpura: A nationwide survey by the Spanish registry of thrombotic thrombocytopenic purpura

Successful Management of Thrombotic Thrombocytopenic Purpura in a Jehovah’s Witness: An Individualized Approach With Joint Decision-Making

Combination of Fresh Frozen Plasma and Cryosupernatant Plasma for Therapeutic Plasma Exchange in Thrombotic Thrombocytopenic Purpura: A Single Institution Experience

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