Use of IDEAL-IQ in Quantifying Femoral Bone Marrow Involvement in Gaucher Disease.

ABSTRACT

OBJECTIVE: To quantitatively measure femoral bone marrow involvement in patients with Gaucher disease (GD) by using fat fraction (FF) derived from the iterative decomposition of water and fat with echo asymmetry and least-squares estimation quantitation (IDEAL-IQ) technique. METHODS: Bilateral femora of 23 patients with type 1 GD receiving low-dose imiglucerase treatment were prospectively scanned using structural magnetic resonance imaging sequences and an IDEAL-IQ sequence. Femoral bone marrow involvement was evaluated by both semiquantification (bone marrow burden [BMB] score based on magnetic resonance imaging structural images) and quantification (FF derived from IDEAL-IQ) methods. These patients were further divided into subgroups according to whether they underwent splenectomy or had bone complications. The interreader agreement of measurements and the correlation between FF and clinical status were statistically analyzed. RESULTS: In patients with GD, both BMB and FF evaluation of femora showed good interreader concordance (intraclass correlation coefficient = 0.98 and 0.99, respectively), and FF highly correlated with BMB score ( P < 0.001). The longer the duration of disease, the lower the FF ( P = 0.026). Femoral FF was lower in subgroups with splenectomy or bone complications than those without splenectomy or bone complications (0.47 ± 0.08 vs 0.60 ± 0.15, 0.51 ± 0.10 vs 0.61 ± 0.17, respectively, both P < 0.05). CONCLUSION: Femoral FF derived from IDEAL-IQ could be used to quantify femoral bone marrow involvement in patients with GD, and low bone marrow FF may predict worse outcomes of GD patients in this small-scale study.