Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2020 May 25;9(5):1601.
doi: 10.3390/jcm9051601.

Development of Algorithm for Clinical Management of Sickle Cell Bone Disease: Evidence for a Role of Vertebral Fractures in Patient Follow-up

Lucia De Franceschi  1 , Daniele Gabbiani  1 , Andrea Giusti  2 , Gianluca Forni  3 , Filippo Stefanoni  1 , Valeria Maria Pinto  3 , Giulia Sartori  1 , Manuela Balocco  3 , Chiara Dal Zotto  1 , Maria Teresa Valenti  1 , Luca Dalle Carbonare  1
Affiliations

Development of Algorithm for Clinical Management of Sickle Cell Bone Disease: Evidence for a Role of Vertebral Fractures in Patient Follow-up

Lucia De Franceschi et al. J Clin Med. .

Abstract

Sickle-cell disease (SCD) is a worldwide distributed hemoglobinopathy, characterized by hemolytic anemia associated with vaso-occlusive events. These result in acute and chronic multiorgan damage. Bone is early involved, leading to long-term disability, chronic pain and fractures. Here, we carried out a retrospective study to evaluate sickle bone disease (SBD) in a cohort of adults with SCD. We assessed bone density, metabolism and turnover. We also evaluated the presence of fractures and the correlation between SCD severity and skeletal manifestations. A total of 71 patients with SCD were analyzed. The mean age of population was 39 ± 10 years, 56% of which were females. We found osteoporosis in a range between 7% and 18% with a high incidence of vertebral fractures. LDH and AST were predictive for the severity of vertebral fractures, while bone density was not. Noteworthy, we identified -1.4 Standard Deviations T-score as the cutoff for detecting the presence of fractures in patients with SCD. Collectively our data allowed us to develop an algorithm for the management of SBD, which may be useful in daily clinical practice to early intersect and treat SBD.

Keywords: Sickle cell disease; bone densitometry; management; osteoporosis; vertebral fractures.

PubMed Disclaimer

Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
Flow-chart of the study. VOC: vaso-occlusive crisis; ACS: acute chest syndrome.
Figure 2
Figure 2
Lumbar bone mineral density grouped according to the World Health Organization (WHO) classification during the study period.
Figure 3
Figure 3
Thoracic x-ray of patients with vertebral fractures (arrows).
Figure 4
Figure 4
ROC (Receiver Operating Characteristic) curve analysis evaluating the sensitivity and specificity of lumbar bone mineral density (BMD) vs presence of fractures in SCD patients.
Figure 5
Figure 5
Algorithm for clinical management of sickle cell bone disease. CTX: carboxy-terminal collagen crosslinks; b-ALP: bone alkaline phosphatase; P1NP: procollagen type 1 N-terminal propeptide; AST: aspartate aminotransferase; LDH: lactate dehydrogenase; DXA: dual x-ray absorptiometry; SCD: sickle cell disease.
See this image and copyright information in PMC

References

    1. Piel F., Steinberg M., Rees D. Sickle Cell Disease. New Engl. J. Med. 2017;376:1561–1573. doi: 10.1056/NEJMra1510865. - DOI - PubMed
    1. Dalle Carbonare L., Matte A., Valenti M.T., Siciliano A., Mori A., Schweiger V., Zampieri G., Perbellini L., De Franceschi L. Hypoxia-reperfusion affects osteogenic lineage and promotes sickle cell bone disease. Blood. 2015;126:2320–2328. doi: 10.1182/blood-2015-04-641969. - DOI - PubMed
    1. Matte A., Recchiuti A., Federti E., Koehl B., Mintz T., El Nemer W., Tharaux P.L., Brousse V., Andolfo I., Lamolinara A., et al. Resolution of sickle cell disease-associated inflammation and tissue damage with 17R-resolvin D1. Blood. 2019;133:252–265. doi: 10.1182/blood-2018-07-865378. - DOI - PMC - PubMed
    1. De Franceschi L., Cappellini M.D., Olivieri O. Thrombosis and sickle cell disease. Semin. Thromb. Hemost. 2011;37:226–236. doi: 10.1055/s-0031-1273087. - DOI - PubMed
    1. Vinchi F., De Franceschi L., Ghigo A., Townes T., Cimino J., Silengo L., Hirsch E., Altruda F., Tolosano E. Hemopexin therapy improves cardiovascular function by preventing heme-induced endothelial toxicity in mouse models of hemolytic diseases. Circulation. 2013;127:1317–1329. doi: 10.1161/CIRCULATIONAHA.112.130179. - DOI - PubMed

LinkOut - more resources