Platelet-specific antibodies and differences in their expression in childhood immune thrombocytopenic purpura predicts clinical progression
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Published on: June 5, 2019
Lingling Fu1, Jie Ma1, Zhengping Cheng1
1Hematology Oncology Center, Beijing Children's Hospital, Capital Medical University Beijing Key Laboratory of Pediatric Hematology Oncology National Key Discipline of Pediatrics, Ministry of Education; MOE Key Laboratory of Major Diseases in Children Beijing China.
Insights
Platelet-specific antibodies can predict childhood immune thrombocytopenic purpura (ITP) progression. Anti-GPIIb/IIIa antibodies indicate a good prognosis, while anti-GPIb/IX antibodies suggest a poor prognosis in ITP patients.
Area of Science:
- Pediatric Hematology
- Immunology
- Clinical Diagnostics
Background:
- Immune thrombocytopenic purpura (ITP) is a common childhood bleeding disorder.
- A significant percentage of childhood ITP cases progress to chronic or refractory forms, impacting quality of life.
- Predictive markers for ITP clinical progression are currently lacking.
Purpose of the Study:
- To evaluate platelet-specific antibodies in children diagnosed with ITP.
- To compare the expression of anti-GPIIb/IIIa and anti-GPIb/IX antibodies.
- To determine if these antibodies can predict clinical progression in childhood ITP.
Main Methods:
- A single-center prospective cohort study involving 134 children initially diagnosed with ITP.
- Assay of anti-GPIIb/IIIa and GPIb/IX antibodies using enzyme-linked immunosorbent assay (ELISA).
- One-year follow-up to analyze antibody expression and clinical prognosis.
Main Results:
- Anti-platelet antibody positivity was observed in 79.8% of cases.
- After one year, 84.3% were newly diagnosed ITP and 13.4% were chronic ITP.
- Anti-GPIIb/IIIa antibodies correlated with a higher likelihood of newly diagnosed ITP, while anti-GPIb/IX antibodies were associated with chronic ITP.
Conclusions:
- Anti-GPIIb/IIIa antibodies (single or double) predict a favorable prognosis in childhood ITP.
- Anti-GPIb/IX antibodies alone predict a poor prognosis.
- Larger, multicenter studies are recommended for confirmation.
Importance:
Immune thrombocytopenic purpura (ITP) is the most common bleeding disorder in children. Despite the highly spontaneously remission, still almost 20% of cases progress into chronic or refractory ITP, which seriously affects children's quality of life. Currently there is no method to predict the initial stage of childhood ITP.
Objectives:
To evaluate platelet-specific antibodies and compare differences in their expression in childhood ITP to predict clinical progression.
Methods:
This is a single-center prospective cohort study from April 2014 to October 2015. We enrolled children initially diagnosed as ITP. Anti-GPIIb/IIIa and GPIb/IX antibodies were assayed by enzyme-linked immunoadsorbent assay (ELISA) and patients were followed up for 1 year. We also analyzed the relationship between the expression of the platelet-specific antibodies GPIIb/IIIa and GPIb/IX and their respective clinical prognoses.
Results:
Overall, 134 cases were enrolled including 77 boys and 57 girls with a median age of 19 months (range: 1 to 159). Positive rates of anti-platelet antibodies were 79.8%. After a 1-year observation period, 84.3% were diagnosed as newly diagnosed ITP and 13.4% were diagnosed as chronic ITP. Patients with anti-GPIIb/IIIa antibody had a higher risk for newly diagnosed ITP compared with patients who were anti-GPIb/IX antibody positive only (93% vs 25%, P = 0.005; 87% vs 25%, P = 0.014, respectively). There were more anti-GPIb/IX antibody positive only cases, diagnosed as chronic ITP, compared with anti-GPIIb/IIIa antibody positive only cases and double GPIIb/IIIa and GPIb/IX antibody positive cases (75% vs 7%, P = 0.005; 75% vs 13%, P = 0.014, respectively). Interpretation.
Interpretation:
Patients with anti-GPIIb/IIIa antibody (either single or double) were predicted to have a good prognosis, whereas anti-GPIb/ IX antibody only predicted a poor prognosis. These results should be confirmed via a larger cohort multicenter study.
