Magnetic resonance imaging of bone marrow changes in Gaucher disease during enzyme replacement therapy: first German long-term results

Since 1991, enzyme replacement therapy (ERT) has been available for patients with Gaucher disease in Germany. The aim of this study was to analyse the MR pattern of bone marrow involvement and response to ERT in Gaucher disease type I. Thirty patients with Gaucher disease type I had MRI examinations...

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Bibliographic Details
Published in:Skeletal radiology 2001-09, Vol.30 (9), p.496-503
Main Authors: Poll, L W, Koch, J A, vom Dahl, S, Willers, R, Scherer, A, Boerner, D, Niederau, C, Häussinger, D, Mödder, U
Format: Article
Language:English
Subjects:
Adult
Aged
Bone marrow
Bone Marrow - drug effects
Bone Marrow - metabolism
Bone Marrow - pathology
Disease
Female
Follow-Up Studies
Gaucher Disease - drug therapy
Gaucher Disease - pathology
Germany
Glucosylceramidase - therapeutic use
Humans
Magnetic Resonance Imaging
Male
Medical research
Middle Aged
Signal Processing, Computer-Assisted
Time Factors
Treatment Outcome
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Summary:Since 1991, enzyme replacement therapy (ERT) has been available for patients with Gaucher disease in Germany. The aim of this study was to analyse the MR pattern of bone marrow involvement and response to ERT in Gaucher disease type I. Thirty patients with Gaucher disease type I had MRI examinations prior to initiation of ERT with alglucerase/imiglucerase and during follow-up. Median MR follow-up and duration of ERT were 36 months. Coronal T1- and T2-weighted spin-echo images of the lower extremities were obtained to evaluate changes in the appearance of yellow marrow. MR images were categorized as having either a homogeneous (type A) or non-homogeneous patchy (type B) appearance of bone involvement and response to ERT was assessed by two radiologists. Overall, 19 of 30 patients (63%) showed an increased signal intensity on T1- and T2-weighted images after 36 months of ERT, consistent with partial reconversion of fatty marrow during treatment. Focal bone lesions surrounded by a low signal intensity (SI) rim did not respond to ERT, suggesting bone infarcts. Of the 11 patients with bone infarcts (low SI rim lesion), 82% had the non-homogeneous type B pattern (P=0.0021). In 86% of patients with splenectomy, bone infarcts were seen (P
ISSN:0364-2348
1432-2161
DOI:10.1007/s002560100375