[MRI bone marrow findings in 63 patients with type I Gaucher disease].
OBJECTIVE
To determine whether MR bone marrow findings in Gaucher patients may help to identify patients at high risk of developing severe Gaucher bone complications exemplified by avascular necrosis (AVN) of the femoral head.


METHODS
MR images were obtained in 63 Type I Gaucher patients through a standard protocol using coronal T 1 and T 2-weighted sequences of the lower extremities. The location and extent of infiltrated marrow was established using a semi-quantitative MRI scoring method (Düsseldorf Gaucher score, DGS) and the morphological pattern of bone marrow involvement determined (whether homogeneous type A or non-homogeneous type B). The active marrow process with bone edema and AVN of the femoral head were also analyzed.


RESULTS
Bone marrow involvement was observed in femoral sites more than in tibial sites. A high DGS was significantly correlated with type B morphology and femoral AVN (both p < 0.0001). Splenectomized patients showed a significantly higher Düsseldorf Gaucher score and type B morphology than non-splenectomized patients (both p < 0.05). AVN was seen in 46 % of patients with type B morphology versus 3 % in type A morphology (p < 0.0001). DGS and morphology of bone marrow involvement were not significantly correlated with active marrow processes.


CONCLUSIONS
Type B marrow morphology and extensive marrow packing were significantly associated with AVN of the femoral head (both p < 0.0001). These patterns are considered predictive and may be employed in a disease management context to alert physicians to the need for urgent therapeutic measures.
Radiologie, Berufsgenossenschaftliche Unfallklinik Duisburg GmbH, Duisburg. lpoll@gmx.de
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Poll
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Willers
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Häussinger
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Mödder
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vom Dahl
D000328:Adult; D000368:Aged; D001853:Bone Marrow / Q000473:pathology*; D004838:Epiphyses / Q000473:pathology; D005260:Female; D005270:Femur Head / Q000473:pathology; D005271:Femur Head Necrosis / Q000175:diagnosis; D005776:Gaucher Disease / Q000175:diagnosis*; D006801:Humans; D007091:Image Processing, Computer-Assisted / Q000379:methods*; D008279:Magnetic Resonance Imaging / Q000379:methods*; D008297:Male; D008875:Middle Aged; D011237:Predictive Value of Tests; D012307:Risk Factors; D012680:Sensitivity and Specificity; D013977:Tibia / Q000473:pathology; D055815:Young Adult