Thrombotic microangiopathy

Mörtzell, Monica; Berlin, Gösta; Nilsson, Thomas; Axelsson, C. G.; Efvergren, M.; Audzijoni, J.; Griskevicius, A.; Pták, Jan; Bláha, M.; Tomsova, H.; Liumbruno, Giancarlo Maria; Centoni, Paolo Emilio; Newman, Elizabeth; Eloot, Sunny; Dhondt, Annemieke; Tomaz, J.; Witt, Volker; Rock, Gail; Stegmayr, Bernd

doi:10.1016/j.transci.2011.07.002

Cited by 8 publications

(14 citation statements)

References 40 publications

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“…Pregnant women and women in the postpartum period accounted for a notable portion (12-31%; about 1 each 25,000 pregnancies) of the cases in some studies. For this reason TTP predominantly affects female subjects between 10 and 40 years old [5,6].…”

Section: Thrombotic Thrombocytopenic Purpura (Ttp)mentioning

confidence: 99%

“…Really, ADAMTS-13 deficiency may vary from 0 to 100% across several studies [2,5,6]. The cause of this variability may reflect differences in ADAMTS-13 assay methods.…”

Section: Thrombotic Thrombocytopenic Purpura (Ttp)mentioning

confidence: 99%

“…The mortality is almost 90% if untreated, but if treated, the mortality is reduced to 15% [6]. Adults and older children with acquired acute idiopathic thrombotic thrombocytopenic purpura require daily plasma exchange [5,6].…”

Section: Thrombotic Thrombocytopenic Purpura (Ttp)mentioning

confidence: 99%

“…Adults and older children with acquired acute idiopathic thrombotic thrombocytopenic purpura require daily plasma exchange [5,6]. Adults and some older children with acquired ADAMTS-13 autoantibody-mediated TTP require daily plasma exchange until remission.…”

Section: Thrombotic Thrombocytopenic Purpura (Ttp)mentioning

confidence: 99%

“…Leukocyte activation and complement consumption are also involved in TMAs pathogenesis [6,7]. Particularly, an increase in vWF have been claimed to account for the loss of physiologic thrombo-resistance and for the consequent widespread platelet aggregation in vascular beds throughout the body, creating a cycle of vasoconstriction with platelet and fibrin deposition and further thrombus formation [3,[6][7][8].…”

Section: Introductionmentioning

confidence: 99%

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Von Willebrand Factor-Mediated Thrombotic Microangiopathies

Gennaro¹,

Lancellotti²,

Cristofaro³

2012

Microangiopathy

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Section: Thrombotic Thrombocytopenic Purpura (Ttp)mentioning

confidence: 99%

“…Really, ADAMTS-13 deficiency may vary from 0 to 100% across several studies [2,5,6]. The cause of this variability may reflect differences in ADAMTS-13 assay methods.…”

Section: Thrombotic Thrombocytopenic Purpura (Ttp)mentioning

confidence: 99%

Section: Thrombotic Thrombocytopenic Purpura (Ttp)mentioning

confidence: 99%

Section: Thrombotic Thrombocytopenic Purpura (Ttp)mentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Von Willebrand Factor-Mediated Thrombotic Microangiopathies

Gennaro¹,

Lancellotti²,

Cristofaro³

2012

Microangiopathy

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A Saudi multicenter experience on therapeutic plasma exchange for patients with thrombotic thrombocytopenic purpura: A call for national registry

Radhwi,

Badawi,

Almarzouki

et al. 2023

J of Clinical Apheresis

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BackgroundThe improvement in the clinical care for patients with thrombotic thrombocytopenic purpura (TTP) is evolving, and many efforts are being put to standardize it. Here, we aimed to assess the provided care at a national level and identify deficiencies.MethodsA national Saudi retrospective descriptive study was carried out at six tertiary referral centers and included all patients who underwent therapeutic plasma exchange (TPE) for the diagnosis of TTP between May 2005, and July 2022. Collected information included demographic data, clinical features on presentation, and the results of laboratory investigations at admission and discharge. In addition, the number of TPE sessions, days till the first session of TPE, usage of immunological agents, and clinical outcomes were all collected.ResultsOne hundred patients were enrolled, predominantly female (56%). The mean age was 36.8 years. At diagnosis, 53% of patients showed neurological involvement. The mean platelet count at presentation was 21 × 109/L. All patients had anemia (mean hematocrit 24.2%). Schistocytes were present in the peripheral blood film of all patients. The mean number of TPE rounds was 13 ± 9.3, and the mean days to start TPE since admission for the first episode was 2.5 days. ADAMTS13 level was measured in 48% of patients and was significantly low in 77% of them. Assessing for clinical TTP scores, 83%, 1000%, 64% of eligible patients had an intermediate/high PLASMIC, FRENCH, and Bentley scores, respectively. Caplacizumab was used on only one patient, and rituximab was administered to 37% of patients. A complete response for the first episode was achieved in 78% of patients. The overall mortality rate was 25%. Neither time to TPE, the use of rituximab or steroid affected survival.ConclusionsOur study shows an excellent response to TPE with a survival rate approximate to the reported international literature. We observed a deficiency in using validated scoring systems in addition to confirming the disease by ADAMTS13 testing. This emphasizes the need for a national registry to facilitate proper diagnosis and management of this rare disorder.

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