Quantification of bone involvement in Gaucher disease: MR imaging bone marrow burden score as an alternative to Dixon quantitative chemical shift MR imaging--initial experience

PURPOSE: To develop a semiquantitative magnetic resonance (MR) imaging bone marrow burden (BMB) score with inclusion of both axial and peripheral bone marrow in Gaucher disease as an alternative to MR imaging with the Dixon quantitative chemical shift imaging (QCSI) technique. MATERIALS AND METHODS: Two experienced musculoskeletal radiologists with no experience in evaluating Gaucher disease blindly analyzed MR images of lumbar spines and femora. Interobserver and intraobserver variability were tested. In addition, the BMB score was determined as a parameter to evaluate bone marrow response to enzyme supplementation therapy. Finally, the BMB score was compared with fat fraction measurements obtained with Dixon QCSI. Differences between groups were analyzed by using the nonparametric Mann-Whitney test. A P value of less than.05 was considered to represent significance. Correlation was calculated by using two-tailed nonparametric rank correlation (Spearman rho). RESULTS: In 30 patients (mean age, 39.3 years; age range, 12-71 years) the mean fat fraction was 0.20 (range, 0.08-0.40). The BMB score range was 3-13 points. A significant correlation was found between the two observers when using BMB (rho = 0.91, P <.001). The intraobserver variation showed a significant correlation (rho = 0.99, P <.001). There was a significant correlation between BMB and QCSI (rho = -0.78, P <.001). Although BMB was less sensitive than Dixon QCSI, it showed enough sensitivity to allow detection of bone marrow response to enzyme supplementation therapy. CONCLUSION: BMB is a reproducible semiquantitative scoring system that is easy to use. It combines MR imaging of both axial and peripheral bone marrow and shows a significant correlation with QCSI.     

Gaucher disease status and treatment assessment: pilot study using magnetic resonance spectroscopy bone marrow fat fractions in pediatric patients

ObjectiveTo assess magnetic resonance spectroscopy (MRS) bone marrow fat fractions' ability to discern between untreated Gaucher disease patients and healthy controls based on assessment of bone marrow infiltration and evaluate response to enzyme replacement therapy (ERT) on serial imaging.MethodsThis retrospective case-controlled study compared conventional MRI and bone marrow MRS findings in six pediatric and young adult Gaucher disease patients with age- and sex-matched controls, examining femoral neck and lumbar spine bone marrow fat fractions and bone marrow burden (BMB) scores. Separate analysis of six patients with serial imaging on ERT was performed with analysis of fat fractions, BMB scores, organ volumes, and serum chitotriosidase.ResultsUntreated patients had significantly lower femoral and lumbar spine fat fractions than controls (0.32 versus 0.67, p = 0.041 and 0.17 versus 0.34, p = 0.041, respectively). Total BMB scores were significantly higher in patients (8.0 versus 3.5, p = 0.015). In patients on ERT with average follow-up of 3.5 years, femoral neck fat fraction was the sole significant predictor of treatment duration (R square: 0.804, p < 0.001) when adjusted for age. Femoral neck fat fraction also correlated with lumbar spine fat fraction, liver volume and chitotriosidase (p < 0.05). MRS test-retest reliability was excellent (Pearson correlations: 0.96, 0.99; p-values <0.001). BMB inter-rater reliability was good overall with an intra-class correlation coefficient of 0.79 for total score, although lumbar spine score reliability was poor at 0.45.ConclusionMRS-derived bone marrow fat fractions appear capable of detecting Gaucher disease severity and monitoring treatment-related changes as a predictor of ERT duration in pediatric and young adult patients.     

全身MRI与PET/CT在淋巴瘤骨髓浸润诊断及预后中的作用

淋巴瘤是一种血液系统恶性肿瘤。淋巴瘤骨髓浸润(BMI)使疾病分期上升至IV期, 是疾病进展、预后较差的标志。常规部位的骨髓活检(BMB)具有创伤性, 且检出率低。PET/CT与全身MRI的出现, 丰富了BMI的检测手段。PET/CT与全身MRI对于淋巴瘤, 尤其是侵袭性淋巴瘤BMI均具有较高的检出率, 二者孰高孰低, 尚未定论。对于红骨髓、良性骨髓病变(炎症等)、淋巴瘤BMI病灶以及肿瘤治疗后骨髓的变化与骨髓残留或复发病灶, 全身MRI很难区分, 而PET/CT却可以很好地鉴别这些病灶。但是, PET/CT存在电离辐射; 对于惰性淋巴瘤的BMI, 超出PET/CT分辨率的病灶, 可能出现假阴性; 某些情况会限制PET/CT的使用, 包括18F-FDG生理性摄取量可能发生改变的正常组织、18F-FDG摄取相关性炎症、高血糖或高胰岛素血症导致的18F-FDG分布的改变、肿瘤患者治疗后出现的骨髓活化等。然而, 这些情况可以使用全身MRI。因此, 全身MRI和PET/CT相辅相成, 优势互补, 但二者均不能代替BMB。对于常规BMB阴性, 但影像学提示阳性的患者, 在影像学引导下进行BMB, 可以提高BMI的检出率。另外, 全身MRI阳性的淋巴瘤BMI患者与全身MRI阴性的淋巴瘤BMI患者相比, 前者预后可能较差。     

Prospective study of bone marrow infiltration in aggressive lymphoma by three independent methods: whole-body MRI, PET/CT and bone marrow biopsy

PURPOSE: Initial lymphoma staging requires bone marrow assessment in aggressive lymphomas. Bone marrow lymphoma infiltration is routinely assessed by bone marrow biopsy (BMB), considered as the "gold standard". The aim of this study was to compare the performance of BMB, whole-body MRI and PET/CT for evaluation of BM infiltration. METHODS: Patients with newly diagnosed aggressive lymphoma were evaluated by BMB, MRI and PET/CT. Two radiologists, two nuclear medicine physicians and one pathologist independently assessed the results of the three modalities. Bone was considered as involved if BM was positive or if PET/CT or MRI was positive and if there was a resolution of the abnormal image shown on PET/CT or MRI halfway or at the end of therapy. RESULTS: Both MRI and PET/CT detected bone marrow lesions in the 9/43 patients, but two patients with multiple lesions had more lesions detected by PET/CT compared to MRI. Among these nine patients, two with an iliac crest lesion detected by both MRI and PET/CT had bone marrow involvement with large-cell lymphoma on histological examination. The other seven patients had focal MRI and PET/CT lesions in areas other than the iliac crest, where the blind BMB was done. The other patients had bone marrow without large-cell lymphoma involvement. In all cases, after lymphoma therapy bone marrow involvement regressed on histological examination, PET and MRI. CONCLUSION: These preliminary results suggest that non-invasive morphological procedures could be superior to BMB for bone marrow assessment in aggressive lymphomas. Ongoing study is underway to validate these results.     

How does iliac crest bone marrow biopsy compare with imaging in the detection of bone metastases in small cell lung cancer?

Iliac crest bone marrow biopsy (BMB) has often been used as the gold standard for the detection of bone marrow metastases in small cell lung cancer (SCLC). However, it is likely to lead to numerous false-negative results. For this reason, we compared the results of bone scintigraphy (BS), magnetic resonance imaging (MRI), and BMB in 48 sequential patients affected with pathologically confirmed SCLC (47 were evaluable; mean age, 58.4 years). The three procedures were carried out within 1 week, no treatment being performed during this period. Whole-body scans and spot views were obtained in the anterior and posterior projections. For MRI, only the thoracolumbar spine, the sternum and the pelvis were scanned, using spin-echo T1-weighted sequences, resulting in an acquisition time of less than 45 min. Only five BMBs were rated as positive. In these cases, both BS and MRI were also positive. The other 42 biopsies were negative. Among them, in ten cases both BS and MRI were positive. In 21 cases, both BS and MRI were negative. In five cases MRI was positive while BS was negative. Finally, in six cases MRI was negative whilst BS was positive. In most cases in which either BS or MRI was positive, follow-up scans confirmed the initial findings. This study suggests that BMB is more invasive and less sensitive than BS or MRI in detecting bone metastases. MRI seems to be more sensitive than BS in detecting small spinal or pelvic metastases. Whole-body bone scintigraphy is more sensitive in detecting skull, costal or peripheral metastases. BS and MRI should be used in combination and may replace BMB in the detection of bone metastases in SCLC. Correspondence to: I. Perrin-Resche     

Bone marrow patterns in aplastic anemia: observations with 1.5-T MR imaging

Six patients diagnosed as having aplastic anemia underwent magnetic resonance (MR) imaging. The bone marrow in the lumbar spine, pelvis, and proximal femora was examined in each case. One patient had a normal marrow signal intensity (SI) and pattern and was considered cured of disease. The other patients had varying numbers of focal low-SI areas interspersed with high-SI areas in the marrow of the spine. The pelves and proximal femora of all patients had diffuse high-SI marrow without focal abnormalities. Biopsy of a lumbar vertebral body in one patient demonstrated that the focal low-SI areas may have been islands of active hematopoietic cells scattered in otherwise fatty marrow. MR may prove to be a valuable, noninvasive method of following the response of patients with aplastic anemia to therapy; more investigation is necessary before this can be definitely concluded.     

18F-FDG PET/CT bone/bone marrow findings in Hodgkin’s lymphoma may circumvent the use of bone marrow trephine biopsy at diagnosis staging

Purpose

Accurate staging of Hodgkin’s lymphoma (HL) is necessary in selecting appropriate treatment. Bone marrow trephine biopsy (BMB) is the standard procedure for depicting bone marrow involvement. BMB is invasive and explores a limited part of the bone marrow. ¹⁸F-FDG PET/CT is now widely used for assessing response to therapy in HL and a baseline study is obtained to improve accuracy. The aim of this retrospective analysis was to assess whether routine BMB remains necessary with concomitant ¹⁸F-FDG PET/CT.

Methods

Data from 83 patients (newly diagnosed HL) were reviewed. All patients had received contrast-enhanced CT, BMB and ¹⁸F-FDG PET/CT. Results of BMB were not available at the time of ¹⁸F-FDG PET/CT imaging.

Results

Seven patients had lymphomatous involvement on BMB. Four patients had bone involvement on conventional CT (two with negative BMB). All patients with bone marrow and/or bone lesions at conventional staging were also diagnosed on ¹⁸F-FDG PET/CT scan. PET/CT depicted FDG-avid bone/bone marrow foci in nine additional patients. Four of them had only one or two foci, while the other had multiple foci. However, the iliac crest, site of the BMB, was not involved on ¹⁸F-FDG PET/CT. Osteolytic/sclerotic lesions matching FDG-avid foci were visible on the CT part of PET/CT in three patients. MRI ordered in three other patients suggested bone marrow involvement. Interim and/or end-therapy ¹⁸F-FDG PET/CT documented response of FDG-avid bone/bone marrow foci to chemotherapy in every patient.

Conclusion

¹⁸F-FDG PET/CT highly improves sensitivity for diagnosis of bone/bone marrow lesions in HL compared to conventional staging.     

The relation of bone marrow scintigraphy and unilateral bone marrow biopsy in malignant lymphoma

To assess the diagnostic role of bone marrow scintigraphy (BMS) for detecting bone marrow infiltration by malignant lymphomas, 47 patients, 14 with malignant Hodgkin's and 33 with non-Hodgkin's lymphoma underwent BMS with 99mTc-sulphur-colloid and also unilateral iliac crest bone marrow biopsy (BMB). BM involvement in BMB was observed in 11 of the 47 patients. Four of these patients also had BMS lesions. Eight patients had BMS lesions not detected by BMB. There was poor agreement between the two modalities (kappa=0.137). Considering BMB as the gold standard, sensitivity, specificity, positive predictive value, negative predictive value and accuracy of BMS were 36%, 77%, 33%, 80%, and 68% respectively. In conclusion, BMS has a high negative predictive value and may be used as a complementary screening test for lymphoma to assess the extent of BM involvement, especially if magnetic resonance imaging-guided biopsy or positron emission tomography studies are not available, as is the case in developing countries.     

Bone involvement in patients with lymphoma: the role of FDG-PET/CT

Purpose To evaluate the diagnostic impact and clinical significance of FDG-avid bone lesions detected by FDG-PET/CT in patients with lymphoma. Methods The study population comprised 50 consecutive patients (mean age 41.7±15.5 years; 27 female, 23 male; 41 staging, 9 restaging) with Hodgkin’s disease (n=22) or aggressive non-Hodgkin’s lymphoma (n=28) in whom FDG-avid bone lesions were detected by FDG-PET/CT. All patients had either direct biopsy of the FDG-avid bone lesion (n=18), standard bone marrow biopsy at the iliac crest (BMB; n=43) or both procedures (n=11). In 15 patients, additional MRI of the bone lesions was performed. All patients underwent FDG-PET/CT after the end of treatment. All CT images of FDG-PET/CT scans were analysed independently regarding morphological osseous changes and compared with FDG-PET results. Results In the 50 patients, 193 FDG-avid lesions were found by PET/CT. The mean standardised uptake value was 6.26 (±3.22). All direct bone biopsies (n=18) of the FDG-avid lesions proved the presence of lymphomatous infiltration. BMB (n=43) was positive in 12 patients (27.9%). In CT, 32 of 193 (16.6%) lesions were detected without the PET information. No additional morphological bone infiltration was detected on CT compared with FDG-PET. All morphological bone alterations on CT scans persisted after the end of therapy. Additional PET/CT information regarding uni- or multifocal bone involvement resulted in lymphoma upstaging in 21 (42%) patients compared with the combined information provided by CT and BMB. Conclusion In patients with FDG-avid bone lesions, FDG-PET is superior to CT alone or in combination with unilateral BMB in detecting bone marrow involvement, leading to upstaging in a relevant proportion of patients.     

多发性骨髓瘤MRI和X线诊断价值

目的　进一步研究多发性骨髓瘤 (multiplemyeloma ,MM )的MRI表现特点并与X线检查相比较其优缺点 ,同时探讨MRI分型与临床分期的联系。材料与方法　选择 19例经骨髓穿刺证实的MM患者的下胸椎至股骨上段行X线与MRI检查 ,对相同患者相同时间相同部位的X线平片与MRI表现、临床分期、MRI分型与骨髓浆细胞百分比进行比较。结果　MM的MRI据T1 WI骨髓信号减低的形态分为以下类型 :正常型 ,弥漫型 ,局灶型 ,混合型 ,“盐和胡椒”型。腰椎以弥漫型多见占 47.3% ,局灶型、混合型各占15 .8% ;骨盆及股骨近段以局灶型多见占 5 7.9% ,弥漫型占 2 1.1% ,无混合型。MM的X线表现 :未见异常 7例 ,骨质疏松的基础上病理性骨折或骨质破坏 12例。腰椎、骨盆及股骨近段各部位检查的阳性率MRI均为 84.2 % ,X线平片分别为 63.2 %、42 .1%和31.6%。MRI表现 :正常型及“盐和胡椒”型的病例其骨髓浸润较轻 ,均见于临床Ⅰ期MM ,其骨髓浆细胞的百分比较低 ( 8.4± 2 .5 ) ;而弥漫型、局灶型、混合型均见于Ⅱ、Ⅲ期MM ,其骨髓浸润程度相对较重 ,骨髓浆细胞的百分比较高 ( 30 .5± 6.7、45 .2± 11.2 )。结论　MM的MRI表现可分为正常型、弥漫型、局灶型、混合型、“盐和胡椒”型。MRI对MM的敏感性明显高于X线检查 ,但两者的特异性较差     

Comparison of Technetium-99m-MIBI imaging with MRI for detection of spine involvement in patients with multiple myeloma

BACKGROUND: Recently, radiopharmaceutical scanning with Tc-99m-MIBI was reported to depict areas with active bone disease in multiple myeloma (MM) with both high sensitivity and specificity. This observation was explained by the uptake of Tc-99m-MIBI by neoplastic cells. The present investigation evaluates whether Tc-99m-MIBI imaging and magnetic resonance imaging (MRI) perform equally well in detecting myelomatous bone marrow lesions. METHODS: In 21 patients with MM, MRIs of the vertebral region TH12 to S1 and whole body scans with Tc-99m-MIBI were done. RESULTS: Tc-99m-MIBI scanning missed bone marrow infiltration in 43 of 87 vertebrae (50.5%) in which MRI showed neoplastic bone marrow involvement. In patients with disease stage I+II, Tc-99m-MIBI scanning was negative in all of 24 vertebrae infiltrated according to MRI. In patients with disease stage III, Tc-99m-MIBI scanning detected 44 of 63 (70%) vertebrae involved by neoplastic disease. CONCLUSION: Tc-99m-MIBI scanning underestimated the extent of myelomatous bone marrow infiltration in the spine, especially in patients with low disease stage.     

Imaging of Gaucher disease

Gaucher disease is the prototypical lysosomal storage disease. It results from the accumulation of undegraded glucosylceramide in the reticuloendothelial system of the bone marrow, spleen and liver due to deficiency of the enzyme glucocerebrosidase. This leads to hematologic, visceral and skeletal maifestions. Build up of glucosylceramide in the liver and spleen results in hepatosplenomegaly. The normal bone marrow is replaced by the accumulating substrate leading to many of the hematologic signs including anemia. The visceral and skeletal manifestations can be visualized with various imaging modalities including radiography, computed tomography, magnetic resonance imaging (MRI) and radionuclide scanning. Prior to the development of enzyme replacement therapy, treatment was only supportive. However, once intravenous enzyme replacement therapy became available in the 1990s it quickly became the standard of care. Enzyme replacement therapy leads to improvement in all manifestations. The visceral and hematologic manifestations respond more quickly usually within a few months or years. The skeletal manifestations take much longer, usually several years, to show improvement. In recent years newer treatment strategies, such as substrate reduction therapy, have been under investigation. Imaging plays a key role in both initial diagnosis and routine monitoring of patient on treatment particularly volumetric MRI of the liver and spleen and MRI of the femora for evaluating bone marrow disease burden.     

Screening for skeletal metastases of the spine and pelvis: gradient echo opposed-phase MRIcompared with bone scintigraphy

Opposed-phase gradient eho (GRE) MRI at 0.5 T was compared with T1-weighted GRE MRI and bone scintigraphy regarding the detection of malignant bone marrow infiltrates of the spine and pelvis. Seventeen control patients and 41 patients with suspected skeletal metastases were studied with plain and gadolinium-enhanced MRI. In the control group only a vertebral haemangiona showed contrast enhancement, while all metastases (confirmed histologically or by follw-up) were enhancing. Opposed-phase surface coil MRI showed a significantly higher contrast-to-noise ratio of 56 metastases than T1-weighted images. In 28 patients body coil opposed-phased MRI detectedmore metastatic foci of the spine and pelvis than did bone scintigraphy (84 vs 56). No scintigraphically visualised lesion was missed by MRI. In conclusion,body coil gadolinium-enhanced opposed-phase GRE MRI may be applied as a screning method for skeletal metastases of the spine and pelvis at intermediate field strengths. Correspondence to: K. Neumann     

Whole-body MRI for the detection of bone marrow involvement in lymphoma: prospective study in 116 patients and comparison with FDG-PET

Objective

To assess and compare the value of whole-body MRI with FDG-PET for detecting bone marrow involvement in lymphoma.

Methods

A total of 116 patients with newly diagnosed lymphoma prospectively underwent whole-body MRI and blind bone marrow biopsy (BMB) of the posterior iliac crest. Of 116 patients, 80 also underwent FDG-PET. Patient-based sensitivities of whole-body MRI for detecting bone marrow involvement were calculated using BMB as reference standard and compared with FDG-PET in aggressive and indolent lymphomas separately.

Results

Sensitivity of whole-body MRI in all lymphomas was 45.5 % [95 % confidence interval (CI): 29.8–62.0 %]. Sensitivity of whole-body MRI in aggressive lymphoma [88.9 % (95 % CI: 54.3–100 %)] was significantly higher (P?=?0.0029) than that in indolent lymphoma [23.5 % (95 % CI: 9.1–47.8 %)]. Sensitivity of FDG-PET in aggressive lymphoma [83.3 % (95 % CI: 41.8–98.9 %)] was also significantly higher (P?=?0.026) than that in indolent lymphoma [12.5 % (95 % CI: 0–49.2 %)]. There were no significant differences in sensitivity between whole-body MRI and FDG-PET (P?=?1.00)

Conclusion

Sensitivity of whole-body MRI for detecting lymphomatous bone marrow involvement is too low to (partially) replace BMB. Sensitivity of whole-body MRI is significantly higher in aggressive lymphoma than in indolent lymphoma and is equal to FDG-PET in both entities.

Key Points

? Bone marrow involvement in lymphoma has prognostic and therapeutic implications. ? Blind bone marrow biopsy (BMB) is standard for bone marrow assessment. ? Neither whole-body MRI nor FDG-PET can yet replace BMB. ? Both techniques have higher sensitivity in aggressive than in indolent lymphoma. ? Both imaging techniques are complementary to BMB.     

Comparative study of fluorodeoxyglucose positron emission tomography and magnetic resonance imaging for the detection of spinal bone marrow infiltration in untreated patients with multiple myeloma

Background: The presence and extent of osteolytic bone lesions in untreated patients with multiple myeloma are important factors in the staging of the disease, and the extent of bone lesions in multiple myeloma cases significantly influences decisions regarding therapy. Recently, fluorodeoxyglucose positron emission tomography (FDG-PET) and magnetic resonance imaging (MRI) have been used to detect bone marrow involvement in patients with multiple myeloma.

Purpose: To compare the efficacy of FDG-PET and MRI for the detection of bone marrow infiltration into the spine in untreated patients with multiple myeloma.

Material and Methods: Twenty-two patients with multiple myeloma underwent both FDG-PET and spine MRI. The examined spinal regions by MRI included 21 thoracic and lumbar spines, one lumbar spine, and 12 cervical spines. The following imaging sequences were performed: T1-weighted spin-echo MRI with and without fat suppression, and T2-weighted spin-echo MRI in the sagittal plane. In the patients with bone marrow abnormalities, an additional contrast-enhanced T1-weighted spin-echo MR image and a fat-suppressed T1-weighted spin-echo MR image were obtained. Patients were divided into three groups on the basis of the criteria defined by Durie and Salmon: stage I (n=9), stage II (n=3), and stage III (n=10). The number and location of lesions detected in both FGD-PET and MRI were recorded, and the lesions were compared using the McNemar test. Bone marrow biopsy results, the patient's clinical examinations, and other imaging findings (MRI, FDG-PET, etc.) were used as references.

Results: In stages I and II (37 lesions in 12 patients), FDG-PET and MRI detected lesions in 78% (29 of 37 lesions) and 86% (32 of 37 lesions), respectively. However, the difference between the abilities of FDG-PET and MRI to detect lesions was not statistically significant (P=0.317). In stage III (101 lesions in 10 patients), FDG-PET and MRI detected lesions in 80% (81 of 101 lesions) and 92% (93 of 101 lesions), respectively. The difference between the abilities of FDG-PET and MRI to detect lesions was statistically significant (P=0.038).

Conclusion: MRI is superior to FDG-PET in detecting bone marrow involvement in the spine of patients with advanced multiple myeloma.     

Apparent diffusion coefficient of the vertebral bone marrow in children with Gaucher's disease type I and III

Purpose

To assess apparent diffusion coefficient (ADC) as a quantitative parameter for detection of vertebral bone marrow infiltration in children with Gaucher’s disease type I and III.

Material and methods

Prospective study was conducted on 20 infants and children (14 M, 6 F; aged 31–61 months; mean age 46 months) with Gaucher’s disease type I (n?=?13) and III (n?=?7), and 20 age and sex matched controls. They underwent routine and diffusion-weighted MR imaging of the lumbar spine using echo planar imaging with b value of 0, 500 and 1000 sec/mm2. The ADC value of the lumbar vertebral bone marrow was compared in different phenotypes and genotypes; and correlated with bone marrow burden score (BMB), chitotriosidase level, hemoglobin and platelet count.

Results

The mean ADC value of marrow infiltration in patients with Gaucher’s disease (0.39?±?0.06?×?10^?3 mm²/s) was significantly lower (P?=?0.001) than that of vertebral bone marrow in controls (0.54?±?0.05?×?10^?3 mm²/s). The cut-off ADC value used to differentiate patients with Gaucher’s disease from controls was (0.47?×?10^?3 mm²/s); with sensitivity of 95 %; specificity of 95 % and area under the curve of 0.986. The L444P/L444P mutation had significantly lower ADC value compared to other mutations (P?=?0.001). The mean ADC value of the bone marrow negatively correlated with BMB (r?=??0.831; P?=?0.001), and biomarkers of disease activity including chitotriosidase (r?=??0.542; P?=?0.014), hemoglobin (r?=??0.727; P?=?0.001) and platelets (r?=??0.698; P?=?0.001).

Conclusion

We concluded that there is significant difference in the ADC value of vertebral bone marrow between children with Gaucher’s disease and controls, and the ADC value correlated well with genotyping and some biomarkers of disease activity.     

Systemic mastocytosis: MRI of bone marrow involvement

Systemic mastocytosis (SM) is an abnormal proliferation of mast cells, located in different structures: skin, bone marrow, spleen, liver and lymph nodes. Magnetic resonance imaging was prospectively performed in ten patients diagnosed by bone marrow biopsy in order to describe the different patterns of bone marrow involvement. Coronal T1-weighted spin-echo images were obtained in vertebral, pelvic, humeral and femoral bones. Depending on the extension of the cell infiltration, three patterns of bone marrow involvement were used: normal/no involvement (N), non-homogeneous (NH) and homogeneous (H). All ten patients presented bone infiltration. The patterns observed were: spine (50 % NH, 50 % H), pelvis (70 % NH), humerus 100(NH) and femur 40 % (NH). T1-weighted MR imaging is a sensitive technique for detecting marrow abnormalities in patients with systemic mastocytosis. There is no correlation between percentage of mast cells in bone marrow biopsy and extent or pattern of bone marrow involvement. Received: 5 June 1998; Revision received: 23 November 1998; Accepted: 15 January 1999     

Paget's diseases of bone: some observations on the relation of the skeletal distribution to pathogenesis

A survey of the prevalence of Paget's disease in the United Kingdom (Barker et al., 1977) revealed 1225 patients with Paget's disease, detected in films of the lumbar spine, pelvis and femoral heads. These films and others obtained in these patients, have been analysed; 100 of the patients received full skeletal surveys. In these, the order of frequency of involvement was pelvis, lumbar spine, sacrum, femur, skull and dorsal spine. A similar distribution was found in those with incomplete surveys, with lower frequencies elsewhere in the skeleton. The extent of disease tended to increase with age. In the pelivs, the femora and the clavicles there was a right-sided bias; in the spine there were well-defined distributional differences. Of the patients with full skeletal surveys, 90% showed the disease in films covering the lumbar spine, pelvis and upper ends of femora and 31% had monostotic disease. The geographical and anatomical findings suggest that environment and stress are factors in the development of Paget's disease.     

成人急性白血病初诊骨髓MRI及临床应用研究

目的：研究成人急性白血病（acute leukemia,AL)患者初诊自然病程状态下骨髓的MRI表现及其临床应用价值。方法：搜经细胞形态学及免疫表型综合分析确诊的成人AL54例,其中髓细胞白血病（AML）28例、急性淋巴细胞白血病（ALL）26例,行骨盆及股骨中上段骨髓MRI,采用SE序列及短时反转恢复（STIR）序列行冠状面扫描,选取15例正常志愿者作为对照组,搜集AL患者同期骨髓象及血常规结果与MRI对比分析。结果：患者骨髓MRI表现按浸润程度和范围分为5级,AML患者23（82％）例属Ⅰ-Ⅲ级病例,2型患者MRI分级总体的分布差异有非常显著性意义（U＝4．233）,P＜0．01）。分别比较2型AL患者各临床诊断指标,显示2屠 骨髓象白血病细胞计数（BMT）、外周血白血病细胞计数（PBT）、外周血白细胞计数（WBC）之间差异有显著性意义（t值分别为4．336、2．008、3．420,P值均＜0．05）。MRI分级与临床诊断指标比较,两者间具有非常显著的一致性（U＝6．099,P＜0．01）。,MRI分级为Ⅳ级的AML患者,缓解率较Ⅰ-Ⅲ级者低（P＜0．05）,MRI分级为Ⅴ级的ALL患者与Ⅳ级患者缓解率的差异无显著性意义（P＞0．1）。结论MRI能反映2型AL不同的骨髓浸润特征,能为AL确诊提供影像学依据。初诊AML患者骨髓MIR对病情的评估,缓解的预测有重要的临床应用价值。     

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

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.