Reconversion of bone marrow in Gaucher disease treated with enzyme therapy documented by MR.
BACKGROUND
Skeletal complications are responsible for significant morbidity in Gaucher patients. Plain radiographs have been unreliable in assessing bone marrow infiltration and activity. A way to assess bone marrow improvement is needed during enzyme therapy.


OBJECTIVE
The purpose of this paper is to assess the usefulness of MR in following improvement of abnormal bone marrow in Gaucher patients on enzyme therapy.


METHODS
Three patients aged 2, 7, and 24 years underwent serial MR scans of the lower extremities before and during treatment with Alglucerase (two patients) and Imiglucerase (one patient). T1-weighted, T2-weighted, STIR and FSE T2-weighted images were utilized. Two patients were imaged after 16 months of therapy, and one patient was imaged after 6 months of therapy.


RESULTS
All patients had improvement in marrow signal consistent with partial reconversion to fatty marrow during treatment. The findings were more marked after prolonged therapy. T1-weighted images demonstrated findings most clearly.


CONCLUSIONS
MR consistently showed improvement in marrow signal in Gaucher patients 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.
Allison
J W
JW
Department of Radiology, Arkansas Children's Hospital, 800 Marshall Street, Slot 105, Little Rock, AR 72202, USA.
James
C A
CA
Arnold
G L
GL
Stine
K C
KC
Becton
D L
DL
Bell
J M
JM
D000328:Adult; D001853:Bone Marrow / Q000187:drug effects / Q000473:pathology*; D002648:Child; D002675:Child, Preschool; D005776:Gaucher Disease / Q000175:diagnosis* / Q000188:drug therapy; D005962:Glucosylceramidase / Q000627:therapeutic use; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging
C070292:alglucerase; D005962:Glucosylceramidase; C090568:imiglucerase