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Assessment of Bone Microarchitecture in Patients with Systemic Mastocytosis and its Association with Clinical and Biochemical Parameters of the Disease

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Abstract

Patients with systemic mastocytosis (SM) are at high risk of bone deterioration. However, the evaluation of bone microarchitecture in this disease remains unclear. We aimed to assess bone microarchitecture in patients with SM. This was a cross-sectional study of 21 adult patients with SM conducted in a quaternary referral hospital in Sao Paulo, Brazil. A healthy, age-, weight-, and sex-matched cohort of 63 participants was used to provide reference values for bone microarchitecture, assessed by high resolution peripheral quantitative computed tomography (HR-pQCT). Total volumetric bone mineral density (vBMD), cortical vBMD, and cortical thickness at the radius were significantly lower in the control group compared with the SM group (all P < 0.001). Patients with aggressive SM had significantly lower trabecular number (Tb.N) (P = 0.035) and estimated failure load (F.load) (P = 0.032) at the tibia compared with those with indolent SM. Handgrip strength was significantly higher in patients who had more Tb.N at the radius (ρ, 0.46; P = 0.036) and tibia (ρ, 0.49; P = 0.002), and lower who had more trabecular separation at the radius (ρ, −0.46; P = 0.035) and tibia (ρ, −0.52; P = 0.016). Strong and positive associations between F.load (ρ, 0.75; P < 0.001) and stiffness (ρ, 0.70; P < 0.001) at the radius, and between F.load at the tibia (ρ, 0.45; P = 0.038) were observed with handgrip strength. In this cross-sectional study, aggressive SM was more susceptible to bone deterioration compared with indolent SM. In addition, the findings demonstrated that handgrip strength was associated with bone microarchitecture and bone strength.

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Funding

This study was supported by Sao Paulo Research Foundation (FAPESP) (grants 19/24782-4).

Author information

Authors and Affiliations

  1. Rheumatology Division, Hospital das Clinicas HCFMUSP, Faculdade de Medicina da Universidade de Sao Paulo, Sao Paulo, Brazil

    Andre S. Franco, Igor H. Murai, Liliam Takayama, Valeria F. Caparbo & Rosa M. R. Pereira

  2. Service of Hematology, Transfusion and Cell Therapy and Laboratory of Medical Investigation in Pathogenesis and Directed Therapy in Onco-Immuno-Hematology (LIM-31) HCFMUSP, University of Sao Paulo Medical School, Sao Paulo, Brazil

    Luan L. Marchi & Elvira D. R. P. Velloso

  3. Hospital Israelita Albert Einstein, Sao Paulo, Brazil

    Elvira D. R. P. Velloso

  4. Faculdade de Medicina, FMUSP da Universidade de São Paulo, Av Dr Arnaldo, 455 – Reumatologia, 3º Andar, Sala 3193, Cerqueira César, Sao Paulo, 01246-903, Brazil

    Andre S. Franco

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  1. Andre S. Franco
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Contributions

ASF had full access to all of the data in the study and take responsibility for the integrity of the data and the accuracy of the data analysis. Concept and design: ASF, IHM, EDRPV, RMRP.Acquisition, analysis, and interpretation: All authors. Drafting of the manuscript: ASF, IHM. Critical revision of the manuscript for important intellectual content: All authors. Statistical analysis: ASF, IHM, EDRPV. Obtained funding: EDRPV, RMRP. Supervision: EDRPV, RMRP. Administrative, technical, or material support: LT, VFC. Dra Luciana Nardinelli (Laboratory of Tumor Biology of Hospital das Clínicas da FMUSP for performing the dPCR KIT D816V studies) and Dr. Elaine Aparecida Rosseto (Clinical Laboratory of Hospital Israelita Albert Einstein) for performing tryptase tests. The authors are thankful to all the patients who participated in this study. All the individuals described in this section received no compensation for their participation in the study.

Corresponding author

Correspondence to Andre S. Franco.

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Conflict of interest

Andre S. Franco, Igor H. Murai, Liliam Takayama, Valeria F. Caparbo, Luan L. Marchi, Elvira D. R. P. Velloso, and Rosa M. R. Pereira declare that they have no conflict of interest.

Role of the Funder/Sponsor

The funders had no role in the design and conduct of the study; collection, management, analysis, and interpretation of the data; preparation, review, or approval of the manuscript; and decision to submit the manuscript for publication.

Human and Animal Rights and Informed Consent

The study was approved by the ethics committee of the Hospital das Clinicas of the School of Medicine of the University of Sao Paulo (#19821519.0.0000.0068, date of approval September 4, 2019) and all the patients provided written informed consent before participation.

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Rosa M. R. Pereira in memoriam.

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Franco, A.S., Murai, I.H., Takayama, L. et al. Assessment of Bone Microarchitecture in Patients with Systemic Mastocytosis and its Association with Clinical and Biochemical Parameters of the Disease. Calcif Tissue Int 113, 276–285 (2023). https://doi.org/10.1007/s00223-023-01107-x

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  • DOI: https://doi.org/10.1007/s00223-023-01107-x

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