Bone marrow relaxation times in Gaucher disease before and after enzyme replacement therapy

Abstract.

The aim of this work was to monitor the effectiveness of enzyme replacement therapy on the basis of the changes in T1 relaxation times in Gaucher patients. A total of 26 patients underwent MR before enzyme replacement therapy; of them, 18 have been followed-up. A total of 22 age-matched controls underwent the same MR study. Scans were focused on the femoral neck, and T1 relaxation times were measured by means of a mixed spin-echo inversion recovery sequence. The T1 relaxation times in Gaucher patients were significantly longer than normal (p < 0.05). After enzyme replacement therapy, T1 relaxation times gradually became closer to those of control subjects, and there was also a significant decrease (p < 0.01) with respect to values before therapy, probably due to an increase in the fat/water ratio. Evaluation of T1 relaxation time may supply a useful indication of Gaucher disease regression after enzyme replacement therapy particularly in those cases in which a normal skeletal appearance corresponds to prolonged T1 relaxation times.