title: Bone marrow response in treated patients with Gaucher disease: evaluation by T1-weighted magnetic resonance images and correlation with reduction in liver and spleen volume.
abstract: OBJECTIVE
  To determine whether T1-weighted magnetic resonance (MR) images can demonstrate response in the marrow of patients with type 1 Gaucher disease treated with enzyme replacement therapy (ERT) and to determine whether a relationship exists between liver and spleen volume reductions and visible marrow changes.
  
  
  METHODS
  Forty-two patients with type 1 Gaucher disease were evaluated on at least two occasions. Thirty-two patients received ERT. Of these patients, 15 had a baseline examination prior to the initiation of ERT. The remaining 10 patients did not receive ERT.
  
  
  METHODS
  T1-weighted and gradient recalled echo (GRE) coronal images of the femurs and hips were obtained. Concurrently, liver and spleen volumes were determined using contiguous breath-hold axial gradient-echo images. T -weighted images of the hips and femurs were evaluated to determine change or lack of change in the yellow marrow.
  
  
  RESULTS
  Of the 32 patients receiving ERT, 14 (44%) demonstrated increased signal on T1-weighted images suggesting an increase in the amount of yellow marrow. If only the 15 patients with a baseline examination were considered, the response rate to ERT was 67%. Using Student's t-test a highly significant correlation (P<0.005) was found between marrow response and reduction in liver and spleen volume.
  
  
  CONCLUSIONS
  Marrow changes in patients receiving ERT can be detected by T -weighted images. This response correlated with reductions in visceral volumes (P<0.0005).
authors: Department of Radiology, University of Southern California School of Medicine, Los Angeles, USA.
authors: M R
authors: MR
authors: Terk
authors: S
authors: S
authors: Dardashti
authors: H A
authors: HA
authors: Liebman
mesh_terms: D000328:Adult; D001853:Bone Marrow / Q000473:pathology*; D005260:Female; D005269:Femur / Q000473:pathology; D005500:Follow-Up Studies; D005776:Gaucher Disease / Q000188:drug therapy* / Q000473:pathology*; D005962:Glucosylceramidase / Q000627:therapeutic use; D006615:Hip / Q000473:pathology; D006801:Humans; D008099:Liver / Q000473:pathology; D008279:Magnetic Resonance Imaging; D008297:Male; D013154:Spleen / Q000473:pathology; D013997:Time Factors
chemical_list: C070292:alglucerase; D005962:Glucosylceramidase; C090568:imiglucerase