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Elevated platelet counts in a cohort of children with moderate-severe osteogenesis imperfecta suggest that inflammation is present
  1. Lois Salter1,2,
  2. Amaka C Offiah1,2,
  3. Nicholas Bishop1,2
  1. 1Academic Unit of Child Health, Department of Oncology and Metabolism, University of Sheffield, Sheffield, UK
  2. 2Sheffield Children’s Hospital, Sheffield, UK
  1. Correspondence to Dr Nicholas Bishop, Academic Unit of Child Health, Department of Oncology and Metabolism, University of Sheffield, Sheffield S10 2TN, UK; n.j.bishop{at}sheffield.ac.uk

Abstract

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.

  • bone disease
  • collagen disorders
  • musculo-skeletal

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Footnotes

  • Contributors NB had the original idea. LS extracted and collated the data. ACO advised on the radiological data and provided expertise in interpretation of images. NB and LS analysed the data. LS, ACO and NB wrote the paper together. NB responded to the reviewers. LS and ACO amended and approved the revision prior to resubmission.

  • Funding LS is an Academic FY2, funded by the National Institutes of Health Research.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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