@article{3124930,
    title = "Evaluation of bone involvement in patients with Gaucher disease: A semi-quantitative magnetic resonance imaging method (using ROI estimation of bone lesion) as an alternative method to semi-quantitative methods used so far",
    author = "Komninaka, V. and Kolomodi, D. and Christoulas, D. and Marinakis, T. and Papatheodorou, A. and Repa, K. and Voskaridou, E. and Revenas, K. and Terpos, E.",
    journal = "European Journal of Haematology",
    year = "2015",
    volume = "95",
    number = "4",
    pages = "342-351",
    publisher = "Wiley-Blackwell Publishing Ltd",
    issn = "0902-4441, 1600-0609",
    doi = "10.1111/ejh.12504",
    keywords = "acid phosphatase tartrate resistant isoenzyme;  carboxy terminal telopeptide;  chitotriosidase;  collagen type 1;  CXCL3 chemokine;  imiglucerase;  biological marker, adolescent;  adult;  aged;  Article;  bone marrow;  bone necrosis;  clinical article;  controlled study;  disease activity;  female;  femur;  Gaucher disease;  human;  lumbar spine;  male;  middle aged;  normal value;  nuclear magnetic resonance imaging;  priority journal;  scoring system;  tibia;  treatment duration;  vision;  bone;  Bone Diseases;  complication;  Gaucher disease;  genetics;  metabolism;  pathology;  procedures;  severity of illness index;  young adult, Adolescent;  Adult;  Aged;  Biomarkers;  Bone and Bones;  Bone Diseases;  Female;  Gaucher Disease;  Humans;  Magnetic Resonance Imaging;  Male;  Middle Aged;  Severity of Illness Index;  Young Adult",
    abstract = "Objective: The aim of this study was to evaluate bone involvement in patients with Gaucher disease (GD) and to propose a novel semi-quantitative magnetic resonance imaging (MRI) staging. Methods: MRI of the lumbar spine, femur, and tibia was performed in 24 patients with GD and 24 healthy controls. We also measured circulating levels of C-C motif ligand-3 (CCL-3) chemokine, C-telopeptide of collagen type-1 (CTX), and tartrate-resistant acid phosphatase isoform type-b (TRACP-5b). Results: We used the following staging based on MRI data: stage I: region of interest (ROI) 1/2 of normal values and bone infiltration up to 30%; stage II: ROI 1/3 of normal values and bone infiltration from 30 to 60%; stage III: ROI 1/4 of normal values and bone infiltration from 60% to 80%; and stage IV: detection of epiphyseal infiltration, osteonecrosis and deformity regardless of the ROI's values. All but two patients had abnormal MRI findings: 9 (37.5%), 6 (25%), 3 (12.5%), and 4 (16.7%) had stages I-IV, respectively. Patients with GD had elevated chitotriosidase, serum TRACP-5b, and CCL-3 levels (P < 0.001). Conclusions: We propose an easily reproducible semi-quantitative scoring system and confirm that patients with GD have abnormal MRI bone findings and enhanced osteoclast activity possibly due to elevated CCL-3. © 2015 John Wiley & Sons A/S."
}