Gaucher disease: assessment with MR imaging.

Lanir, Amos; Hadar, Herzlia; Cohen, Ian T.; Tal, Yuval; Benmair, J.; Schreiber, R.; Clouse, M E

doi:10.1148/radiology.161.1.3763873

Cited by 57 publications

(26 citation statements)

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“…The epiphysis, however, was involved in six of the 16 patients in group B and seven of the nine patients in group C. In general, our findings correspond with those of Lanir et al [15] and Rosenthal et al [16], who reported that the epiphyses were generally spared unless the involvement of bone was extensive.…”

Section: (Arrows)supporting

confidence: 91%

“…Some patients may have a diffuse infiltration and others may have a patchy infiltration, with islands of preserved fatty marrow. This hypothesis would explain why some researchers observed a homogeneous change in signal intensity [15] while others observed a non-homogeneous pattern [16]. We observed minor variations in the homogeneity but did not further classify these variations into subgroups.…”

Section: (Arrows)contrasting

confidence: 62%

“…The signal intensity of the involved marrow in Gaucher disease has been reported to be homogeneously or nonhomogeneously decreased [5,15,16]. We observed minor variations in the homogeneity of the signal intensity within the marrow.…”

Section: (Arrows)mentioning

confidence: 46%

“…This reduction is due to the deposition of glucocerebrosides in the marrow in Gaucher disease. Gaucher-cell-infiltrated bone marrow also contains protein, glycoproteins, and other components which may contribute to the decreased signal intensity of the marrow on both TI-and T2-weighted images [15].…”

Section: (Arrows)mentioning

confidence: 99%

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MR imaging in adults with Gaucher disease type I: evaluation of marrow involvement and disease activity

et al. 1993

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An investigation was conducted to determine the usefulness of magnetic resonance imaging (MRI) in the evaluation of bone marrow involvement in patients with Gaucher disease type I. T1- and T2-weighted images were obtained of the lower extremities of 29 adult patients. Patients were classified into one of three groups based on marrow signal patterns on T1- and T2-weighted images as well as change in signal intensity from T1- to T2-weighted images. An increase in signal intensity from T1- to T2-weighted images was the criterion for an "active process" within the bone marrow. Classification of the 29 patients produced the following results: group A: normal, 4 patients; group B: marrow infiltration, 16 patients; group C: marrow infiltration plus active marrow process, 9 patients. Correlation with clinical findings revealed that all nine patients with evidence of an active marrow process on MRI (group C) had acute bone pain. Conversely, only one of the remaining 20 patients (groups A and B) had bone pain. There was no correlation between disease activity and findings on conventional radiographs. We conclude the MRI provides an excellent noninvasive assessment of the extent and activity of marrow involvement in type I Gaucher disease.

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Section: (Arrows)supporting

confidence: 91%

Section: (Arrows)contrasting

confidence: 62%

Section: (Arrows)mentioning

confidence: 46%

Section: (Arrows)mentioning

confidence: 99%

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MR imaging in adults with Gaucher disease type I: evaluation of marrow involvement and disease activity

et al. 1993

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“…Infiltration and replacement of the hematopoietic marrow by fibrotic tissue results in a lowering of marrow signal intensity on MRI of all conventional sequence types [34,35]. The diminished signal intensity on T2-weighted images is a feature that is also observed in Gaucher's disease [36]. In preleukemic conditions, MRI contributes to the assessment of the prognosis.…”

Section: Myelofibrosis and Other Related Diseasesmentioning

confidence: 99%

Magnetic Resonance Imaging of the Bone Marrow: Malignant Lymphoma – Multiple Myeloma – Bone Marrow

Golder¹

1999

Oncol Res Treat

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Magnetic resonance imaging (MRI) has become the most applied imaging technique in evaluating lymphomatous diseases, multiple myeloma, myelodysplastic syndromes, and gross therapeutic effects in bone marrow. The tomograms help explain the etiology of pain, palsy, and other clinical symptoms in patients with hematologic malignancies by elucidating osseous and extra-osseous components in their relationships to nerves and vessels. Moreover, MRI permits the detection of bone marrow abnormalities before skeletal lesions become evident on plain radiographs, but it cannot reliably predict the histological nature of a marrow lesion, just like bone marrow scintigraphy. Almost every third of the patients who have had negative bone marrow biopsies for malignant lymphoma, will be recognized as suffering from marrow involvement on a subsequent MR imaging procedure. Consequently, the clinical stage IV will be assigned to a remarkable percentage of patients on the basis of MRI results. In multiple myeloma, MRI has proved to be very reliable for depicting all types of marrow involvement. To assess the local extent of the tumor and to search for other thus far hidden foci of the disease, the technique has become an integral part of the staging procedure in patients with solitary plasmacytoma. All three fundamental MR patterns of abnormal bone marrow have been observed in patients with multiple myeloma, the focal one being most frequent. The prognostic significance of the different MR patterns is controversial. MRI identifies patients at high risk for the development of vertebral collapses, but cannot predict the time and precise level of the fracture. The criteria defined for differentiating benign and malignant vertebral collapses at MRI are quite reliable. Quantitative MRI seems to enable monitoring of treatment response. The typical fatty appearance of the irradiated spine is irreversible for doses exceeding 30 Gy and serves as a dependable indicator for the extent of the irradiation field. MRI can direct harvest procedures for autologous bone marrow transplantation to sites of relatively unaffected marrow. In the evaluation of the status of bone marrow after transplantation, quantitative contrast-enhanced MRI seems to be a promising new tool, because the degree and rate of gadolinium chelate uptake depends on the cellularity of the marrow.

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Bone density in type 1 gaucher disease

Pastores

Wallenstein

Desnick

et al. 1996

Journal of Bone and Mineral Research

100

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Skeletal involvement is a major source of complications in patients with Type 1 Gaucher disease. To investigate the bone density and potential usefulness of bone densitometry in Gaucher disease, dual-energy X-ray absorptiometry was used to measure the density of the lumbar spine, femoral neck, trochanter, and distal radius in 61 adult patients ranging in age from 22 to 77 years. The mean bone density at each site was significantly lower than expected for age and sex. The severity of the osteopenia correlated significantly with other clinical indicators of disease severity, including the N370S/84GG genotype, prior splenectomy, and hepatomegaly. The bone density measurements also correlated significantly with the severity of skeletal disease as assessed by skeletal radiography. Vertebral density remained an independent predictor of the severity of bone involvement even after controlling for age, sex, weight, genotype, splenectomy, and hepatomegaly. These findings suggest that bone density measurements provide a quantitative assessment of bone involvement in Type 1 Gaucher disease, which may permit serial, noninvasive monitoring of bone changes in this progressive disorder.

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Gaucher disease: assessment with MR imaging.

Cited by 57 publications

References 0 publications

MR imaging in adults with Gaucher disease type I: evaluation of marrow involvement and disease activity

MR imaging in adults with Gaucher disease type I: evaluation of marrow involvement and disease activity

Magnetic Resonance Imaging of the Bone Marrow: Malignant Lymphoma – Multiple Myeloma – Bone Marrow

Bone density in type 1 gaucher disease

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