Pediatric thrombotic thrombocytopenic purpura

Joly, Bérangère S.; Coppo, Paul; Veyradier, Agnès

doi:10.1111/ejh.13107

Cited by 53 publications

(74 citation statements)

References 64 publications

(148 reference statements)

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“…The incident rate was similar to those reported locally. Studies suggested that patients with TTP-SLE often had moderate to severe lupus disease activity [13], and this is in accordance with the data we observed in this study (average SLEDAI = 22.4).…”

Section: Discussionsupporting

confidence: 93%

“…In our research, a 15 years old patient with 13 SLEDAI score who had received glucocorticoid therapy combined with plasma exchange to achieve disease remission, and the number of plasma exchanges was small (4 times). While the combined therapies (plasma exchange and immunosuppressor including steroids and cytotoxic agents) were found to be more appropriate than plasma exchange or immunosuppressor alone in treating adult SLE-TTP [13]. This case suggests that for patients with older age and stable disease, we can try plasma exchange combined with glucocorticoid therapy firstly.…”

Section: Discussionmentioning

confidence: 99%

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Clinical features and prognosis of patients with thrombotic thrombocytopenic purpura associated with systemic lupus erythematosus: a review of 25 cases

Jiang

Wang

et al. 2019

Ital J Pediatr

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Objective To report the clinical features of patients with systemic lupus erythematosus (SLE) associated with thrombotic thrombocytopenic purpura (TTP). Their diagnosis, treatment, and prognosis were also discussed. Methods A total of 25 TTP-SLE pediatric patients were included in this study. Their clinical symptoms, laboratory findings, disease activity, and renal biopsy were retrospectively reviewed. Results The median age of the patient cohort was 14 years old. Nine patients were first diagnosed with SLE, followed by the diagnosis of TTP-SLE, whereas 15 patients were diagnosed with TTP and SLE concurrently. All the 25 TTP-SLE patients had decreased platelet count and microangiopathic hemolytic anemia. Fever, rash, edema and neurological symptoms were the main clinical symptoms. Fragmentation of erythrocytes on blood smear and increased LDH were found in all patients. Nineteen patients (76%) had impaired renal function. Renal biopsy showed that most of the patients had lupus nephritis class IV (20%) and TMA (20%). 13 patients (52%) were treated with glucocorticoids in combination with immunosuppressive agent, and 10 patients (40%) were treated with plasma exchange combined with glucocorticoids plus immunosuppressive agent. One patient died due to lung infection; others had disease remission. Fifteen patients had follow-up regularly, and their conditions were stable. Conclusion Patients with TTP-SLE often had moderate to severe lupus disease activity. Testing of LDH level and blood smear should be performed when kidney and neurological symptoms arise in children with SLE. The use of combination therapy, glucocorticoids plus immunosuppressive agent, provided satisfactory clinical outcome. Patients with refractory TTP-SLE will also need plasma exchange therapy.

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Section: Discussionsupporting

confidence: 93%

Section: Discussionmentioning

confidence: 99%

Clinical features and prognosis of patients with thrombotic thrombocytopenic purpura associated with systemic lupus erythematosus: a review of 25 cases

Jiang

Wang

et al. 2019

Ital J Pediatr

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“…One patient died due to lung infection; others had disease remission. These data showed that TTP-SLE often had a moderate to severe lupus disease activity, as confirmed in literature [114]. Testing of LDH level and blood smear should be performed when kidney and neurological symptoms arise in children with SLE.…”

Section: Rheumatic Diseasessupporting

confidence: 81%

Advances in paediatrics in 2019: current practices and challenges in allergy, endocrinology, gastroenterology, public health, neonatology, nutrition, nephrology, neurology, respiratory diseases and rheumatic diseases

et al. 2020

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We highlight the main developments that have been published during the first semester of the last year in the Italian Journal of Pediatrics. We have carefully chosen information from numerous exciting progresses issued in the Journal in the field of allergy, endocrinology, gastroenterology, neonatology, nutrition, nephrology, neurology, public health, respiratory diseases and rheumatic diseases. The impact on the care of patients has been placed in the broader context of studies that appeared in other journals. We think that many observations can be used directly to upgrade management of patients.

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“…This category of endothelial dysfunction results from various imbalances between platelet, endothelial, immune system, and cytokine production (2). Thrombotic thrombocytopenic purpura (TTP) is a rare fatal condition among hematological diseases characterized by microvascular thrombosis with platelet aggregation in a patient with severe functional de ciency of ADAMTS13 (activity < 10%) (3).…”

Section: Introductionmentioning

confidence: 99%

Thrombotic thrombocytopenic purpura associated with COVID-19 in a critically ill child: A Peruvian case report

Domínguez-Rojas

Campano

Tasayco

et al. 2021

Preprint

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Background Acquired thrombotic thrombocytopenic purpura (TTP) is a rare disease. This pathology requires necessary resources for its diagnosis, especially in the context of the pandemic by the new SARS-CoV-2 coronavirus due to the lack of diagnostic tests and the variable response to plasma exchange in middle and low-income countries. Case report: We report a case of a 9-year-old Hispanic boy with SARS-CoV-2 (IgG) infection, with atypical presentation and multisystemic involvement, thrombotic microangiopathy, dermal manifestations, hematologic, kidney, and neurologic involvement. Post SARS-CoV-2 infection evolution, application of the PLASMIC score, and genetic study were performed. Ventilatory and hemodynamic support, corticotherapy, immunoglobulins, plasma exchange, renal replacement therapy, and monoclonal antibodies were given without favorable response. Conclusions TTP associated with SARS-CoV-2 is infrequent in the pediatric population. Necessary resources are needed to make the diagnosis and maintain the necessary support and management to avoid fatal outcomes.

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Pediatric thrombotic thrombocytopenic purpura

Cited by 53 publications

References 64 publications

Clinical features and prognosis of patients with thrombotic thrombocytopenic purpura associated with systemic lupus erythematosus: a review of 25 cases

Clinical features and prognosis of patients with thrombotic thrombocytopenic purpura associated with systemic lupus erythematosus: a review of 25 cases

Advances in paediatrics in 2019: current practices and challenges in allergy, endocrinology, gastroenterology, public health, neonatology, nutrition, nephrology, neurology, respiratory diseases and rheumatic diseases

Thrombotic thrombocytopenic purpura associated with COVID-19 in a critically ill child: A Peruvian case report

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