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Chronic childhood idiopathic thrombocytopenia purpura: long-term follow-up
H Tamary1, C Kaplinsky, I Levy
1Sambur Center for Pediatric Hematology-Oncology, Children's Medical Center of Israel, Beilinson Medical Campus, Petah Tiqva, Israel.
Insights
Chronic childhood idiopathic thrombocytopenia purpura (ITP) often has a mild course. Most children achieve remission with supportive care, suggesting conservative treatment is best.
Area of Science:
- Pediatrics
- Hematology
- Immunology
Background:
- Childhood chronic idiopathic thrombocytopenia purpura (ITP) treatment remains controversial.
- Understanding ITP's natural history is crucial for effective therapeutic strategies.
Purpose of the Study:
- To investigate the natural history and treatment outcomes of childhood chronic ITP.
- To evaluate the efficacy of different therapeutic approaches in managing chronic ITP.
Main Methods:
- Retrospective study of 92 children with ITP, focusing on 22 with a chronic course.
- Follow-up duration ranged from 3 to 14 years (median 8.6 years).
- Individualized treatment included observation, steroids, IVIg, splenectomy, and immunosuppressive therapy.
Main Results:
- 63.6% achieved complete remission, 22.7% partial remission; only 13.5% remained severely thrombocytopenic.
- 50% initially responded to prednisone, but platelet counts decreased upon steroid tapering.
- The majority of patients experienced a mild disease course with minimal bleeding.
Conclusions:
- Childhood chronic ITP generally follows a mild course with high remission rates.
- Conservative management with adequate supportive therapy minimizes complications and fatalities.
- Aggressive treatments like splenectomy should be reserved for rare, severe cases.
Abstract:
An understanding of the natural history of childhood chronic idiopathic thrombocytopenia purpura (ITP) could contribute to a rational therapeutic approach to its treatment, which remains controversial. In our retrospective study of 92 children with ITP, 22 had a chronic course and were followed for 3-14 years (median 8.6 years). Treatment, when indicated, was individualized: 4 patients (18.2%) did not receive any treatment, 14 (63.6%) received steroids only, while 4 (18.2%) were treated with steroids and one of the following: high-dose gamma globulin (4 patients), splenectomy (2 patients) or immunosuppressive therapy (2 patients). During follow-up, 14 patients (63.6%) achieved complete remission, 5 (22.7%) partial remission and only 3 (13.5%) remained severely thrombocytopenic, with minimal bleeding tendency. Eleven patients (50%) responded to the initial prednisone course (1-5 mg/kg/day), but showed a marked decrease in platelet count when steroids were tapered off. In view of the high rates of complete and partial remission and the mild course of the few non-responding patients, it is suggested that with adequate supportive therapy, follow-up problems and fatalities can be kept to a minimum. We believe that aggressive therapy, such as splenectomy, should be reserved for the rare symptomatic and severely thrombocytopenic patient.