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Idiopathic thrombocytopenic purpura in Egyptian children
A S Khalifa1, K A Tolba, M S el-Alfy
1Department of Pediatrics, Faculty of Medicine, Ain Shams University, Cairo, Egypt.
Insights
Idiopathic thrombocytopenic purpura (ITP) in children is often benign, with acute forms responding well to high-dose methylprednisolone or IV immunoglobulin. Chronic ITP may require further treatment, but fatality is not reported.
Area of Science:
- Pediatric Hematology
- Immunology
- Clinical Medicine
Background:
- Idiopathic thrombocytopenic purpura (ITP) is a significant hemorrhagic diathesis in children.
- Understanding the presentation, course, and therapeutic responses in pediatric ITP is crucial for effective management.
- This study retrospectively analyzed a large cohort of pediatric ITP patients.
Purpose of the Study:
- To evaluate the clinical characteristics, treatment outcomes, and prognostic factors of pediatric idiopathic thrombocytopenic purpura.
- To compare the efficacy of different therapeutic regimens for acute and chronic ITP.
- To assess the long-term course and potential complications of ITP in children.
Main Methods:
- Retrospective analysis of 350 pediatric patients with ITP diagnosed between 1975 and 1992.
- Patients were categorized into acute, chronic, and recurrent forms.
- Therapeutic interventions included high-dose methylprednisolone (HDMP), intravenous immunoglobulin (IVIG), conventional-dose prednisone (CDP), vincristine, and splenectomy, with response assessment based on platelet count and clinical improvement.
Main Results:
- Acute ITP was the predominant form (71.4%), often preceded by viral illness, particularly in winter/spring.
- HDMP and IVIG showed dramatic responses in acute ITP with low platelet counts.
- Conventional-dose prednisone achieved complete response in 30% of chronic ITP cases; splenectomy yielded a 50% complete response rate in refractory cases.
Conclusions:
- Pediatric ITP is generally a benign condition with no reported fatalities in this cohort.
- Acute ITP typically resolves, while chronic forms can be refractory, necessitating diverse therapeutic strategies.
- Early and appropriate treatment, including HDMP, IVIG, and potentially splenectomy, can improve outcomes in pediatric ITP.
Abstract:
350 patients with idiopathic thrombocytopenic purpura (ITP) aged 2/12-15 years (mean 6.3 +/- 2.7) were followed up during the period January 1st, 1975 to March 31, 1992. They constituted 40% of cases with hemorrhagic diathesis attending the Hematology/Oncology Clinic, Children's Hospital, Ain Shams University (relative frequency of 37.4/100.000 of the general Out-Patient Clinic in the same hospital). These patients presented with acute (71.4%), chronic (22.9%) and recurrent (5.7%) forms. The age of presentation was younger in acute ITP. In the recurrent form there was significant female predominance. Most cases of acute ITP (66%) presented in winter and spring, with a positive history of preceding viral illness in 50% in contrast to 10% in chronic form. Four chronic ITP cases developed lupus erythematosus; all were females > 9 years. As regards therapy, acute ITP cases with initial platelet count (PC) < 10 x 10(9)/l were randomized to receive either high-dose methyl prednisolone (HDMP) 10 mg/kg/day for 5 days i.v. (n = 10) or intravenous immunoglobulin (IVIG) 0.4 g/kg/day for 5 days (n = 10) or conventional-dose prednisone (CDP) 2 mg/kg/day 4 weeks p.o. (n = 10). A dramatic response was noticed in the first two groups. In chronic ITP, (n = 80) CDP induced complete response (CR) in 30% and partial response (PR) in 20%; 50% were nonresponders. Twenty-four refractory ITP with persistent PC < or = 20 x 10(9)/l received second-line therapy: vincristine 1.5 mg/m2/week i.v. 4 doses (n = 4) with no clinical or hematological improvement. IVIG 0.4 g/kg/day for 5 days (n = 8) with sustained CR only in 2 patients (25%) and PR in 2 patients (25%). Splenectomy was performed (n = 12) with CR in 50%; out of them, 2 patients had shown no improvement on prior IVIG therapy. In conclusion, ITP is a benign condition with no fatality reported, but it could run a chronic refractory course.