More about low-dose rituximab and plasma exchange as front-line therapy for patients with thrombotic thrombocytopenic purpura

Vazquez-Mellado, Alberto; Pequeño-Luévano, Myrna; Cantú-Rodrı́guez, Olga Graciela; Villarreal‐Martínez, Laura; Jaime‐Pérez, José Carlos; Gómez-De-León, Andrés; Garza-Salazar, Fernando De la; González-Llano, Óscar; Colunga-Pedraza, Perla R.; Sotomayor-Duque, Guillermo; Gómez‐Almaguer, David

doi:10.1080/10245332.2015.1133008

Cited by 24 publications

(17 citation statements)

References 24 publications

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“…However, most developing countries cannot afford specialized studies to identify this potentially fatal disease. There is scant literature reporting the experience of Latin‐American countries on TTP, so little is known about its prevalence, clinical course, and outcomes in this region . According to our data, approximately three newly TTP cases per million people are diagnosed in Mexico each year.…”

Section: Discussionmentioning

confidence: 83%

“…In addition, we must remark that TPE after admission was not initiated early in all patients, with a median elapsed time of 2 days, perhaps due to the lack of clinical suspicion and/or availability of the apheresis machines. Furthermore, 15 of our patients also received low‐dose rituximab (100 mg weekly), which has been shown to reduce the risk of relapse; and 12 of these patients achieved clinical response (80%). Treatment with low‐dose rituximab was more frequently used than steroids, being the latter more common in other studies .…”

Section: Discussionmentioning

confidence: 99%

“…this region. [14][15][16] According to our data, approximately three newly TTP cases per million people are diagnosed in Mexico each year. As aforementioned, TTP is more common in young patients, and females are more frequently affected than males.…”

Section: Discussionmentioning

confidence: 99%

“…37,38 In Mexico, as well as other Latin-American countries, there are few publications describing the clinical characteristics and laboratory findings in patients diagnosed with TTP; hence, epidemiological information related to this disease is not well known in this region. [14][15][16] Our review of the literature showed that in developing countries, diagnosis of TTP is largely based on clinical presentation; the presence of thrombocytopenia, anemia associated with peripheral schistocytes, and elevated serum LDH is highly suggestive of the disease, without the need for expensive molecular tests. Therapeutic plasma exchange is initiated in these settings, and improvement of symptoms, elevation of platelet counts, and decrease in serum LDH support the diagnosis.…”

Section: Discussionmentioning

confidence: 99%

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Clinical presentation in thrombotic thrombocytopenic purpura: Real‐world data from two Mexican institutions

Bugarin-Estrada

León

Lopez-Garcia

et al. 2018

J of Clinical Apheresis

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Background Thrombotic thrombocytopenic purpura (TTP) is a hematologic disease that can be fatal if not treated early. We aimed to describe the clinical characteristics of Mexican patients with idiopathic TTP. Study design and methods We conducted a retrospective study, including all adult patients diagnosed with idiopathic TTP from 2011 to 2017 in two Mexican centers. We further compared our results with the published literature. Results Twenty patients were included; 70% were female, with a median age of 38.5 years at diagnosis (range 16‐63). The median time from onset of symptoms to hospital admission was 1.5 days (range 0‐16). Most patients (85%) presented with at least one systemic manifestation at admission (including fever) and 90% had neurological symptoms, most of them major (70%) including loss of consciousness, transient focal abnormalities, headache, and confusion. Only one patient (5%) had the classical pentad at the time of admission. Kidney failure was present in 25% of patients and hemorrhagic symptoms in 60%. Digestive and cardiorespiratory symptoms were less common (45% and 15%, respectively). Median platelet count and lactate dehydrogenase were 10 500/μL and 1319 IU/L, respectively. Eighty percent of patients achieved remission following treatment. Patients admitted within the first 48 hours (after the onset of symptoms) tended to have better overall survival. Conclusion Clinical presentation in Mexican TTP patients is similar to that in other countries. Early admission and a high suspicion for the disease will avoid delays in the initial work‐up and initiation of therapy, further improving prognosis.

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Section: Discussionmentioning

confidence: 83%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Clinical presentation in thrombotic thrombocytopenic purpura: Real‐world data from two Mexican institutions

Bugarin-Estrada

León

Lopez-Garcia

et al. 2018

J of Clinical Apheresis

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“…Vazquez-Mellado et al [50] ont montré une efficacité similaire à la posologie de 100 mg par semaine pendant 4 semaines. Le rituximab pourrait diminuer le risque de rechutes [51].…”

Section: Traitement Du Pttunclassified

Micro-angiopathies thrombotiques du péripartum : physiopathologie, diagnostic et traitement

Vigneron¹,

Hertig²

2017

Méd. Intensive Réa

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Même en contexte de grossesse physiologique, un placenta produit en grande quantité des protéines antiangiogéniques et des hormones stéroïdes, qui circulent dans le sang de la mère, et modifient considérablement le « milieu » auquel son endothélium est exposé. Si la placentation est défectueuse, la pré-éclampsie peut induire jusqu'à une micro-angiopathie thrombotique, localisée dans le foie maternel : c'est le HELLP syndrome. Dans les cas où une défaillance rénale ou neurologique est associée, ou encore si la cytolyse hépatique manque au tableau, débute alors une procédure de diagnostic différentiel pour ne pas méconnaître deux pathologies plus rares, mais qui ne guériront pas spontanément après la délivrance, et qui ne sont même pas, en soi, des indications à la délivrance : le syndrome hémolytique et urémique, et le purpura thrombotique thrombocytopénique. La grossesse en est en effet une circonstance de révélation. L'objectif de cette revue est de discuter, dans ce contexte, les mécanismes de ces différentes formes de microangiopathies thrombotiques, et de proposer au réanimateur une démarche à la fois diagnostique et thérapeutique. Mots clés Micro-angiopathies thrombotiques · PéripartumAbstract Even in the context of physiological pregnancy, a placenta produces large amounts of anti-angiogenic proteins and steroid hormones, which circulate in the mother's blood and greatly alters the "environment" to which its endothelium is exposed. If the placentation is defective, preeclam-psia can induce up to thrombotic micro-angiopathy, localized in the maternal liver: this is the HELLP syndrome. In cases where renal or neurological failure is associated, or if hepatic cytolysis is missing in the table, then a differential diagnosis procedure is initiated in order not to ignore two pathologies that are rarer, but which will not cure spontaneously after delivery, and which are not, in themselves, indications for delivery: hemolytic and uremic syndrome and thrombotic thrombocytopenic purpura. Pregnancy is indeed a circumstance of revelation. The objective of this review is to discuss the mechanisms of these different forms of thrombotic micro-angiopathy in the context of pregnancy and to propose to the intensivist a diagnostic and therapeutic approach.

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Thrombotic Thrombocytopenic Purpura

1962

JAMA

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Thrombotic thrombocytopenic purpura (TTP) is a disease with a high rate of mortality if a proper treatment is not instated. Plasmapheresis with plasmatic exchange is the treatment of choice. Diagnosis is performed demonstrating microangiopathic hemolytic anemia, a negative direct Coombs test and thrombocytopenia. Among the clinical data, neurological and renal alterations stand out. When there is a reasonable suspicion in the diagnosis, plasmapheresis must be initiated immediately. There are different diseases that may be similar to the TTP signs and symptoms, especially in pregnant women. TTP has a high risk of relapse and may leave sequelae.

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More about low-dose rituximab and plasma exchange as front-line therapy for patients with thrombotic thrombocytopenic purpura

Abstract: Our study suggests that low-dose rituximab and PEx are effective as front-line treatment for acute TTP; however, a prospective trial is needed to demonstrate whether low-dose rituximab is as effective as the conventional dose.

Cited by 24 publications

References 24 publications

Clinical presentation in thrombotic thrombocytopenic purpura: Real‐world data from two Mexican institutions

Clinical presentation in thrombotic thrombocytopenic purpura: Real‐world data from two Mexican institutions

Micro-angiopathies thrombotiques du péripartum : physiopathologie, diagnostic et traitement

Thrombotic Thrombocytopenic Purpura

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