RECONVERSION OF BONE-MARROW IN GAUCHER-DISEASE TREATED WITH ENZYME THERAPY DOCUMENTED BY MR

Background Skeletal complications are responsible for significant morb idity in Gaucher patients. Plain radiographs have been unreliable in a ssessing bone marrow infiltration and activity. A way to assess bone m arrow improvement is needed during enzyme therapy. Objective. The purp ose of this paper is to assess the usefulness of MR in following impro vement of abnormal bone marrow in Gaucher patients on enzyme therapy. Materials and methods. Three patients aged 2, 7, and 24 years underwen t serial MR scans of the lower extremities before and during treatment with Alglucerase (two patients) and Imiglucerase (one patient). T1-we ighted, T2-weighted, STIR and FSE T2-weighted images were utilized. Tw o patients were imaged after 16 months of therapy, and one patient was imaged after 6 months of therapy. Results. All patients had improveme nt in marrow signal consistent with partial reconversion to fatty marr ow during treatment. The findings were more marked after prolonged the rapy. T1-weighted images demonstrated findings most clearlyConclusion. MR consistently showed improvement in marrow signal in Gaucher patien ts on enzyme therapy. As smaller doses of enzyme therapy are the trend , MR can be utilized to determine if therapy is effecting a change in the bone marrow.