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Cited by 12Mast cells and histamineBone health and osteoporosis researchHematological disorders and diagnostics

Underlying systemic mastocytosis in patients with unexplained osteoporosis: score proposal

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Tanasi, Ilaria, Crosera, Lara, Taus, Francesco, Orsolini, Giovanni, Adami, Giovanni, Olivieri, Francesco, Bernardelli, Andrea, Bonadonna, Patrizia +11 more·2024·Bone
DOI 10.1016/j.bone.2024.117141PMC PMC11854854
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Abstract

Background A score to predict the association between unexplained osteoporosis and an underlying systemic Mastocytosis (SM) is lacking. Objective This study aimed at identifying criteria able to predict the diagnosis of SM without skin involvement and provide an indication for bone marrow (BM) assessment. Methods We included 139 adult patients with unexplained osteoporosis and suspected SM. After BM evaluation, 63 patients (45.3 %) were diagnosed with SM, while the remaining 76 patients (54.7 %) negative for clonal mast cell (MC) disorders, constituted our control group. Univariate and multivariate analysis identified three independent predictive factors: age (<54 years: +1 point, >64 years: -1 point), serum basal tryptase (sBT) levels >19 ng/mL (+2 points) and vertebral fractures (+2 points). Results These variables were used to build the OSTEO-score, able to predict the diagnosis of SM before BM assessment with a sensitivity of 73.5 % and a specificity of 67.1 %. Patients with a score < 3 had a lower probability of having SM compared to patients with a score ≥ 3 (28.5 % and 71.4 %, respectively, p < 0.0001). When sBT levels were corrected for the presence of hereditary alpha-tryptasemia (HαT) using the BST calculater (https://bst-calculater.niaid.nih.gov/) recently published [1,2], the sensitivity of ΗαT-adjusted OSTEO-score increased to 87.8 %, and the specificity reached 76.1 %. Also, the positive predictive value of a score ≥ 3 increased to 85.2 %. Conclusions Further studies are needed to validate these results and characterize the role of tryptase genotyping in patients with unexplained osteoporosis in reducing the risk of misdiagnosing patients with SM. Our proposed scoring model allows the identification of patients with the highest probability of having SM, avoiding unnecessary BM studies.

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  • The OSTEO score predicts the risk of systemic mastocytosis in patients with osteoporosis, including evaluation of basal serum tryptase.

    “Predicts the risk of SM in patient with osteoporosis including evaluation of BST.”

    From Defining “Normal” basal serum tryptase levels: a context-dependent approach to improve diagnostics in systemic mastocytosis (2025) · cites this paper as Tanasi et al., 2024

    The Abstract Methods and Results describe an OSTEO score that uses basal serum tryptase to predict an SM diagnosis. The studied population was adults with unexplained osteoporosis and suspected SM, not patients with osteoporosis generally, so the claim broadens its scope. (LLM, conf=0.84)

    Experimental — accuracy not yet measured

  • These data have justified the development of a score specific for this setting (OSTEO-score) with increased sensitivity when BST levels were corrected for the presence of HαT.

    “These data have justified the development of a score specific for this setting (OSTEO-score) with increased sensitivity when BST levels were corrected for the presence of HαT (69).”

    From Defining “Normal” basal serum tryptase levels: a context-dependent approach to improve diagnostics in systemic mastocytosis (2025) · cites this paper as [69]

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References 33 · Cited by 12

Research works printed but not linked: 23

  1. [2]Lyons JJ, Greiner G, Hoermann G, Metcalfe DD, Incorporating Tryptase Genotyping Into the Workup and Diagnosis of Mast Cell Diseases and Reactions, J Allergy Clin Immunol Pract 10 (2022) 1964– 1973. 10.1016/j.jaip.2022.05.003. 35597543 PMC11837873 · DOI
  2. [4]Orsolini G, Viapiana O, Rossini M, Bonifacio M, Zanotti R, Bone Disease in Mastocytosis, Immunol Allergy Clin North Am 38 (2018) 443– 454. 10.1016/j.iac.2018.04.013. 30007462 · DOI
  3. [5]Rossini M, Zanotti R, Viapiana O, Tripi G, Orsolini G, Idolazzi L, Bonadonna P, Schena D, Escribano L, Adami S, Gatti D, Bone involvement and osteoporosis in mastocytosis, Immunol Allergy Clin North Am 34 (2014) 383– 396. 10.1016/j.iac.2014.01.011. 24745681 · DOI
  4. [6]Barete S, Assous N, de Gennes C, Grandpeix C, Feger F, Palmerini F, Dubreuil P, Arock M, Roux C, Launay JM, Fraitag S, Canioni D, Billemont B, Suarez F, Lanternier F, Lortholary O, Hermine O, Francès C, Systemic mastocytosis and bone involvement in a cohort of 75 patients, Ann Rheum Dis 69 (2010) 1838– 1841. 10.1136/ard.2009.124511. 20570833 · DOI
  5. [7]van der Veer E, van der Goot W, de Monchy JGR, Kluin-Nelemans HC, van Doormaal JJ, High prevalence of fractures and osteoporosis in patients with indolent systemic mastocytosis, Allergy 67 (2012) 431– 438. 10.1111/j.1398-9995.2011.02780.x. 22229787 · DOI · Paper page
  6. [8]Rossini M, Zanotti R, Orsolini G, Tripi G, Viapiana O, Idolazzi L, Zamò A, Bonadonna P, Kunnathully V, Adami S, Gatti D, Prevalence, pathogenesis, and treatment options for mastocytosis-related osteoporosis, Osteoporos Int 27 (2016) 2411– 2421. 10.1007/s00198-016-3539-1. 26892042 · DOI
  7. [9]Makovoz A, Wang J, Oshegbo G, Park YH, Lyons JJ, Eisch AR, Scott LM, Reynolds JC, Ortega-Villa AM, Metcalfe DD, Komarow HD, Assessment of Osteoporosis and Fracture Risk in Mastocytosis within a North American Cohort, J Allergy Clin Immunol Pract 9 (2021) 4459– 4467.e10. 10.1016/j.jaip.2021.08.001. 34403839 · DOI
  8. [10]Gehlen M, Schmidt N, Pfeifer M, Balasingam S, Schwarz-Eywill M, Maier A, Werner M, Siggelkow H, Osteoporosis Caused by Systemic Mastocytosis: Prevalence in a Cohort of 8392 Patients with Osteoporosis, Calcif Tissue Int 109 (2021) 685– 695. 10.1007/s00223-021-00887-4. 34223956 · DOI
  9. [11]Alvarez-Twose I, González-de-Olano D, Sánchez-Muñoz L, Matito A, Jara-Acevedo M, Teodosio C, García-Montero A, Morgado JM, Orfao A, Escribano L, Validation of the REMA Score for Predicting Mast Cell Clonality and Systemic Mastocytosis in Patients with Systemic Mast Cell Activation Symptoms, IAA 157 (2012) 275– 280. 10.1159/000329856. 22042301 · DOI · Paper page
  10. [16]Sordi B, Vanderwert F, Crupi F, Gesullo F, Zanotti R, Bonadonna P, Crosera L, Elena C, Fiorelli N, Ferrari J, Grifoni F, Sciumè M, Parente R, Triggiani M, Palterer B, Mecheri V, Almerigogna F, Santi R, Di Medio L, Brandi ML, Iorno ML, Ciardetti I, Bencini S, Annunziato F, Mannarelli C, Pieri L, Guglielmelli P, Mannelli F, Vannucchi AM, Disease correlates and clinical relevance of hereditary α-tryptasemia in patients with systemic mastocytosis, J Allergy Clin Immunol (2022) S0091–6749(22)01424–5. 10.1016/j.jaci.2022.09.038. 36309122 · DOI · Paper page
  11. [18]Zanotti R, Tanasi I, Bernardelli A, Orsolini G, Bonadonna P, Bone Marrow Mastocytosis: A Diagnostic Challenge, J Clin Med 10 (2021) 1420. 10.3390/jcm10071420. 33915965 PMC8037514 · DOI
  12. [19]Arber DA, Orazi A, Hasserjian R, Thiele J, Borowitz MJ, Beau MML, Bloomfield CD, Cazzola M, Vardiman JW, The 2016 revision to the World Health Organization classification of myeloid neoplasms and acute leukemia, Blood 127 (2016) 2391– 2405. 10.1182/blood-2016-03-643544. 27069254 · DOI · Paper page
  13. [20]Khoury JD, Solary E, Abla O, Akkari Y, Alaggio R, Apperley JF, Bejar R, Berti E, Busque L, Chan JKC, Chen W, Chen X, Chng W-J, Choi JK, Colmenero I, Coupland SE, Cross NCP, De Jong D, Elghetany MT, Takahashi E, Emile J-F, Ferry J, Fogelstrand L, Fontenay M, Germing U, Gujral S, Haferlach T, Harrison C, Hodge JC, Hu S, Jansen JH, Kanagal-Shamanna R, Kantarjian HM, Kratz CP, Li X-Q, Lim MS, Loeb K, Loghavi S, Marcogliese A, Meshinchi S, Michaels P, Naresh KN, Natkunam Y, Nejati R, Ott G, Padron E, Patel KP, Patkar N, Picarsic J, Platzbecker U, Roberts I, Schuh A, Sewell W, Siebert R, Tembhare P, Tyner J, Verstovsek S, Wang W, Wood B, Xiao W, Yeung C, Hochhaus A, The 5th edition of the World Health Organization Classification of Haematolymphoid Tumours: Myeloid and Histiocytic/Dendritic Neoplasms, Leukemia 36 (2022) 1703– 1719. 10.1038/s41375-022-01613-1. 35732831 PMC9252913 · DOI · Paper page
  14. [21]Arock M, Sotlar K, Akin C, Broesby-Olsen S, Hoermann G, Escribano L, Kristensen TK, Kluin-Nelemans HC, Hermine O, Dubreuil P, Sperr WR, Hartmann K, Gotlib J, Cross NCP, Haferlach T, Garcia-Montero A, Orfao A, Schwaab J, Triggiani M, Horny H-P, Metcalfe DD, Reiter A, Valent P, KIT mutation analysis in mast cell neoplasms: recommendations of the European Competence Network on Mastocytosis, Leukemia 29 (2015) 1223– 1232. 10.1038/leu.2015.24. 25650093 PMC4522520 · DOI · Paper page
  15. [22]Gotlib J, Gerds AT, Bose P, Castells MC, Deininger MW, Gojo I, Gundabolu K, Hobbs G, Jamieson C, McMahon B, Mohan SR, Oehler V, Oh S, Padron E, Pancari P, Papadantonakis N, Pardanani A, Podoltsev N, Rampal R, Ranheim E, Rein L, Snyder DS, Stein BL, Talpaz M, Thota S, Wadleigh M, Walsh K, Bergman MA, Sundar H, Systemic Mastocytosis, Version 2.2019, NCCN Clinical Practice Guidelines in Oncology, J Natl Compr Canc Netw 16 (2018) 1500– 1537. 10.6004/jnccn.2018.0088. 30545997 · DOI · Paper page
  16. [23]Kristensen T, Vestergaard H, Bindslev-Jensen C, Møller MB, Broesby-Olsen S, Mastocytosis Centre, Odense University Hospital (MastOUH), Sensitive KIT D816V mutation analysis of blood as a diagnostic test in mastocytosis, Am J Hematol 89 (2014) 493– 498. 10.1002/ajh.23672. 24443360 · DOI
  17. [25]Anderson PA, Freedman BA, Brox WT, Shaffer WO, Osteoporosis: Recent Recommendations and Positions of the American Society for Bone and Mineral Research and the International Society for Clinical Densitometry, J Bone Joint Surg Am 103 (2021) 741– 747. 10.2106/JBJS.20.01248. 33587517 · DOI
  18. [26]Valent P, Akin C, Hartmann K, Alvarez-Twose I, Brockow K, Hermine O, Niedoszytko M, Schwaab J, Lyons JJ, Carter MC, Elberink HO, Butterfield JH, George TI, Greiner G, Ustun C, Bonadonna P, Sotlar K, Nilsson G, Jawhar M, Siebenhaar F, Broesby-Olsen S, Yavuz S, Zanotti R, Lange M, Nedoszytko B, Hoermann G, Castells M, Radia DH, Muñoz-Gonzalez JI, Sperr WR, Triggiani M, Kluin-Nelemans HC, Galli SJ, Schwartz LB, Reiter A, Orfao A, Gotlib J, Arock M, Horny H-P, Metcalfe DD, Updated Diagnostic Criteria and Classification of Mast Cell Disorders: A Consensus Proposal, Hemasphere 5 (2021) e646. 10.1097/HS9.0000000000000646. 34901755 PMC8659997 · DOI · Paper page
  19. [27]Sullivan LM, Massaro JM, D’Agostino RB, Presentation of multivariate data for clinical use: The Framingham Study risk score functions, Stat Med 23 (2004) 1631– 1660. 10.1002/sim.1742. 15122742 · DOI
  20. [30]van der Veer E, Arends S, van der Hoek S, Versluijs JB, de Monchy JGR, Oude Elberink JNG, van Doormaal JJ, Predictors of new fragility fractures after diagnosis of indolent systemic mastocytosis, J Allergy Clin Immunol 134 (2014) 1413– 1421. 10.1016/j.jaci.2014.05.003. 24985401 · DOI
  21. [31]Guillaume N, Desoutter J, Chandesris O, Merlusca L, Henry I, Georgin-Lavialle S, Barete S, Hirsch I, Bouredji D, Royer B, Gruson B, Lok C, Sevestre H, Mentaverri R, Brazier M, Meynier J, Hermine O, Marolleau J-P, Kamel S, Damaj G, Bone complications of mastocytosis: a link between clinical and biological characteristics, Am J Med 126 (2013) 75.e1–7. 10.1016/j.amjmed.2012.07.018. 23200108 · DOI
  22. [32]Navarro-Navarro P, Álvarez-Twose I, Pérez-Pons A, Henriques A, Mayado A, García-Montero AC, Sánchez-Muñoz L, González-López O, Matito A, Caldas C, Jara-Acevedo M, Orfao A, KITD816V mutation in blood for the diagnostic screening of systemic mastocytosis and mast cell activation syndromes, Allergy 78 (2023) 1347– 1359. 10.1111/all.15584. 36385619 · DOI · Paper page
  23. [33]Polivka L, Rossignol J, Neuraz A, Condé D, Agopian J, Méni C, Garcelon N, Dubreuil P, Maouche-Chrétien L, Hadj-Rabia S, Hermine O, Bodemer C, Criteria for the Regression of Pediatric Mastocytosis: A Long-Term Follow-Up, The Journal of Allergy and Clinical Immunology: In Practice 9 (2021) 1695– 1704.e5. 10.1016/j.jaip.2020.12.019. 33338682 · DOI

Authors
Tanasi, I., Crosera, L., Taus, F., Orsolini, G., Adami, G., Olivieri, F., Bernardelli, A., Bonadonna, P., Nalin, F., Sella, S., Giannini, S., Liu, Y., Mannelli, F., Vanderwert, F., Bonifacio, M., Krampera, M., Rossini, M., Lyons, J. J., & Zanotti, R.
Affiliations
  • Azienda Ospedaliera Universitaria Integrata Verona (IT)
  • University of Verona (IT)
  • University of Padua (IT)
  • National Institutes of Health (US)
  • University of Florence (IT)
Corresponding author
Tanasi, Ilaria
ORCID
Venue
Bone
Publisher
Elsevier BV
Published
2024-09
Volume
186
Pages
117141
ISSN
  • 1873-2763 (ISSN-L)
  • 8756-3282
DOI
10.1016/j.bone.2024.117141
PMC
PMC11854854
OpenAlex
W4399244639
Cited by
12
References
33
Citations by year
2026: 4 · 2025: 8
FWCI
2.01
Type
journal article
Language
en
Open access
Licence
Locations
  • Bone (publishedVersion)
  • PubMed Central (publishedVersion)
  • PubMed (publishedVersion)
  • Europe PMC (PubMed Central) (acceptedVersion)
  • Florence Research (University of Florence) (submittedVersion, other-oa)
  • Padua Research Archive (University of Padova) (submittedVersion)
Funders
  • National Institute of Allergy and Infectious Diseases
  • U.S. Department of Health and Human Services
  • National Institutes of Health
  • National Institute of Allergy and Infectious Diseases Division of Intramural Research
Topics
  • Life Sciences › Immunology and Microbiology › Immunology › Mast cells and histamine
  • Health Sciences › Medicine › Orthopedics and Sports Medicine › Bone health and osteoporosis research
  • Health Sciences › Medicine › Emergency Medicine › Hematological disorders and diagnostics
Keywords
systemic mastocytosis, predictive score, serum tryptase, hereditary alpha-tryptasemia, vertebral fractures, clonal mast cell disorders, sensitivity and specificity
MeSH terms
Adult, Aged, Bone Marrow, Female, Humans, Male, Middle Aged, Osteoporosis, Mastocytosis, Systemic, Tryptases

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