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Elevated platelet counts in a cohort of children with moderate-severe osteogenesis imperfecta suggest that
Lois Salter1,2, Amaka C Offiah1,2, Nicholas Bishop1,2
1Academic Unit of Child Health, Department of Oncology and Metabolism, University of Sheffield, Sheffield, UK.
Insights
Children with osteogenesis imperfecta (OI) often have elevated platelet counts, even without clear inflammation. This suggests a potential inflammatory component in OI that may be a therapeutic target.
Area of Science:
- Pediatric Hematology
- Genetics and Genetic Diseases
- Inflammation and Immunology
Background:
- Elevated platelet counts are linked to cancer, autoimmunity, and inflammation.
- Proinflammatory cytokines are elevated in mouse models of osteogenesis imperfecta (OI).
- This study investigates elevated platelet counts in children with moderate-to-severe OI.
Purpose of the Study:
- To determine if children with moderate-to-severe osteogenesis imperfecta (OI) exhibit elevated platelet counts.
- To explore the relationship between platelet counts, age, OI type, and concurrent health events.
Main Methods:
- Retrospective review of hospital records for 71 children with moderate-to-severe OI.
- Analysis of 1206 platelet counts, assessing counts relative to average and upper limits.
- Calculation of platelet standard deviation (SD) scores to evaluate age and time-related trends.
Main Results:
- 69% of patients had at least one high platelet count, with 20.4% of all counts exceeding the normal range.
- 41% of elevated counts occurred without identifiable confounding factors.
- Elevated platelet counts were most frequent in children with new or existing vertebral fractures.
Conclusions:
- Raised platelet counts are associated with fractures in OI patients.
- A significant proportion of elevated platelet counts in OI children occur without apparent inflammation, suggesting an intrinsic proinflammatory component.
- This potential proinflammatory aspect of OI warrants further investigation as a therapeutic target.
Background:
Elevated platelet counts are observed in cancer, autoimmunity and inflammation with concurrent illness. Proinflammatory cytokines are elevated in murine osteogenesis imperfecta (OI) models. We hypothesised that platelet counts might be elevated in children with moderate-severe OI.
Methods:
We reviewed the hospital records of 71 children with moderate-severe OI, treated in the Sheffield Children's Hospital's Severe, Complex and Atypical Osteogenesis Imperfecta Highly Specialised Service. Data relating platelet count (below/above average, above upper limit) to prior and concurrent events were summarised as event proportions per child. Additionally, we created platelet SD scores to assess age and time-related trends, and relationship with OI type.
Results:
1206 platelet counts were recorded. Platelet SD scores were right-shifted by 0.89 SD overall. 49 of 71 (69%) patients had at least one platelet count above the normal range and 246 (20.4%) of all counts were above the upper limit of normal. Of these, 101 (41%) were high despite no confounding factors being present. For the 47 children with data at age less than 2 years, 89 (30.0%) platelet counts were above the upper limit of normal and 39 (44%) had no associated confounding factor. Elevated platelet counts were recorded most often for children with new or existing vertebral fractures.
Conclusions:
Raised platelet counts were observed in association with new and healing vertebral fractures, but also (41%-44%) in the absence of identified proinflammatory factors or events. We speculate that these findings are evidence for a proinflammatory component to OI that could be a target for therapeutic intervention.
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