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. 2024 Nov;47(11):2775-2784.
doi: 10.1007/s40618-024-02365-8. Epub 2024 Apr 1.

Analysis of circulating osteoclast and osteogenic precursors in patients with Gorham-Stout disease

M Rossi  1 , S Terreri  1 , G Battafarano  1 , I Rana  2 , P S Buonuomo  2 , L Di Giuseppe  3 , M D'Agostini  4 , O Porzio  4   5 , J Di Gregorio  6 , Cristiana Cipriani  3 , A Jenkner  7 , M V Gonfiantini  2 , A Bartuli  2 , A Del Fattore  8
Affiliations

Analysis of circulating osteoclast and osteogenic precursors in patients with Gorham-Stout disease

M Rossi et al. J Endocrinol Invest. 2024 Nov.

Abstract

Purpose: Gorham-Stout disease is a very rare disorder characterized by progressive bone erosion and angiomatous proliferation; its etiopathogenesis is still unknown, and diagnosis is still performed by exclusion criteria. The alteration of bone remodeling activity has been reported in patients; in this study, we characterized circulating osteoclast and osteogenic precursors that could be important to better understand the osteolysis observed in patients.

Methods: Flow cytometry analysis of PBMC (Peripheral Blood Mononuclear Cells) was performed to characterize circulating osteoclast and osteogenic precursors in GSD patients (n = 9) compared to healthy donors (n = 55). Moreover, ELISA assays were assessed to evaluate serum levels of bone markers including RANK-L (Receptor activator of NF-κB ligand), OPG (Osteoprotegerin), BALP (Bone Alkaline Phosphatase) and OCN (Osteocalcin).

Results: We found an increase of CD16-/CD14+CD11b+ and CD115+/CD14+CD11b+ osteoclast precursors in GSD patients, with high levels of serum RANK-L that could reflect the increase of bone resorption activity observed in patients. Moreover, no significant alterations were found regarding osteogenic precursors and serum levels of BALP and OCN.

Conclusion: The analysis of circulating bone cell precursors, as well as of RANK-L, could be relevant as an additional diagnostic tool for these patients and could be exploited for therapeutic purposes.

Keywords: Diseases and disorders related to bone; Gorham-stout disease; Osteoclast precursors; Osteoclasts.

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References

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