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Eur J Haematol. 2015 Oct;95(4):342-51. doi: 10.1111/ejh.12504. Epub 2015 Mar 23.

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 information

1
Thalassaemia Centre, Laikon General Hospital, Athens, Greece.
2
Department of Radiology, Laikon General Hospital, Athens, Greece.
3
Department of Haematology, 251 General Air-Force Hospital, Athens, Greece.
4
Department of Haematology, "Georgios Gennimatas" General Hospital, Athens, Greece.
5
Department of Medical Research, 251 General Air-Force Hospital, Athens, Greece.
6
Department of Clinical Therapeutics, National and Kapodistrian University of Athens, School of Medicine, Athens, Greece.

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.

KEYWORDS:

C-C motif ligand-3 (CCL-3) chemokine; Gaucher disease; bone markers; bone marrow involvement; magnetic resonance imaging

PMID:
25645321
DOI:
10.1111/ejh.12504
[Indexed for MEDLINE]

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