Idiopathic thrombocytopenic purpura in childhood: twenty years of experience in a single center.

Autor: Stiakaki E; Department of Pediatric Hematology-Oncology, University of Crete, Heraklion, Crete, Greece., Perdikogianni C, Thomou C, Markaki EA, Katzilakis N, Tsirigotaki M, Kalmanti M
Jazyk: angličtina
Zdroj: Pediatrics international : official journal of the Japan Pediatric Society [Pediatr Int] 2012 Aug; Vol. 54 (4), pp. 524-7. Date of Electronic Publication: 2012 May 30.
DOI: 10.1111/j.1442-200X.2012.03606.x
Abstrakt: Background: Idiopathic thrombocytopenic purpura (ITP) is an autoimmune disorder with a variable clinical course.
Methods: A retrospective analysis was carried out of ITP patients presenting to a pediatric hematology-oncology department during a period of 20 years, with a focus on treatment and outcome.
Results: One hundred and twenty-four cases were recorded (mean patient age, 8.4 years). Forty-nine children (39.5%) had platelet counts <10,000/µL at diagnosis. No episode of severe bleeding was observed. Peak incidence was observed during spring and summer. Respiratory infections proceeded in 58% of cases. Treatment consisted of i.v. immunoglobulin (IVIG) in 93 children at four dosing schedules. Sixteen children received corticosteroids, 10 children received anti-D immunoglobulin and 14 received no treatment. Recovery was observed in 67% of children on IVIG and in 50% on anti-D globulin. Eight patients did not respond initially and received corticosteroids. Three children with refractory thrombocytopenia received anti-CD20 (rituximab). Fourteen children (11%) had persistent/chronic disease. In 10 of them recovery was observed in 13 months-8 years. Splenectomy was performed in six children with resistant/chronic disease.
Conclusion: ITP has a benign course in the majority of cases. Anti-D globulin can effectively be used as an alternative first-line treatment. Rituximab can successfully be used in refractory cases, while splenectomy has currently limited indications.
(© 2012 The Authors. Pediatrics International © 2012 Japan Pediatric Society.)
Databáze: MEDLINE