Thrombotic thrombocytopenic purpura in pediatric patients
DOI:
https://doi.org/10.35954/SM2025.44.2.5.e501Keywords:
anemis, hemolytic, ADAMTS-13 protein, purpura, thrombotic thrombocytopenic, thrombocytopeniaAbstract
Introduction: Thrombotic thrombocytopenic purpura is a clinical syndrome of high mortality and very low prevalence, with a frequency of less than 5%, even lower in pediatric age. It presents with microangiopathic hemolytic anemia, thrombocytopenia, neurological symptoms, fever and renal involvement.
Objective: To report on a rare pathology in childhood, whose low prevalence and given its presentation requires a high diagnostic suspicion for its detection.
Clinical case: Male patient, 11 years old, healthy. He consults for easy bruising of 2 weeks of evolution, with no other history of bleeding or fever. Physical examination revealed: right malar ecchymosis of 1 centimeter, petechiae and ecchymosis in the right iliac crest and lower limbs. No neurofocal elements. No digestive transit alterations. On admission: hemoglobin 9.6 g/dl, platelet count in lamina less than 40,000/mm³, total bilirubin normal; lactate dehydrogenase 585 IU; negative direct Coombs test.
Because the peripheral lamina showed 5% of schistocytes and increased total bilirubin with indirect predominance, PLASMIC Score was applied, which showed intermediate risk for thrombotic thrombocytopenic purpura, and this diagnosis was considered. ADAMTS-13 and ADAMTS-13 inhibitor screening were dosed, and without waiting for results, daily therapeutic plasma exchanges were started using fresh plasma as replacement fluid.
A total of 22 therapeutic plasma exchanges were performed, 4 doses of rituximab and daily corticoids, with good clinical-paraclinical evolution and no relapse to date.
Conclusion: The low prevalence of thrombotic thrombocytopenic purpura in pediatric age makes its diagnosis difficult, but the use of PLASMIC Score allows us to determine high risk patients early, for a quick start of specific treatment in a pathology with a high mortality rate without timely treatment.
Note: This article was approved by the Editorial Committee.
Received for review: April 2025.
Accepted for publication: June 2025.
Correspondence: 8 de Octubre 3020, C.P. 11100. Tel.: (+598) 24876666 ext. 1998. Montevideo, Uruguay.
Contact email: rosariogomezrearden@gmail.com
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