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Radiographic and MRI Features of Deferiprone-Related Arthropathy of the Knees in Patients with ß-Thalassemia

Christian J. Kellenberger1,2, Markus Schmugge3, Traudel Saurenmann3, Lin Di Gennaro3, Stefan W. Eber3, Ulrich V. Willi1 and Paul S. Babyn2

1 Department of Diagnostic Imaging and Radiology, University Children's Hospital, Steinwiesstrasse 75, Zurich CH-8032, Switzerland.
2 Present address: Department of Diagnostic Imaging, The Hospital for Sick Children, 555 University Ave., Toronto, ON M5G 1X8, Canada.
3 Division of Haematology and Immunology, University Children's Hospital, Zurich CH-8032, Switzerland.



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Fig. 1A. 18-year-old man with ß-thalassemia major who was receiving chelation therapy with L1 (patient 5 in Tables 1 and 2). Mild radiographic changes were detected. Patient had experienced several episodes of severe knee pain with swelling that eventually led to discontinuation of L1 therapy. Frontal radiograph of left knee shows mild irregular subchondral cortical flattening of femoral condyles (arrows).

 


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Fig. 1B. 18-year-old man with ß-thalassemia major who was receiving chelation therapy with L1 (patient 5 in Tables 1 and 2). Mild radiographic changes were detected. Patient had experienced several episodes of severe knee pain with swelling that eventually led to discontinuation of L1 therapy. Lateral radiograph of left knee reveals broad patellar beak (arrowhead) in addition to irregularities of subchondral bone (arrows).

 


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Fig. 2A. 10-year-old boy with ß-thalassemia major who was receiving chelation therapy with L1 (patient 6 in Tables 1 and 2). Moderate radiographic and severe MRI changes were seen at initial study. Patient had moderate knee pain at time of initial imaging, and subsequent episodes of severe arthralgia led to discontinuation of L1 therapy. Lateral radiograph of right knee shows moderate irregular subchondral cortical flattening of femoral condyles.

 


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Fig. 2B. 10-year-old boy with ß-thalassemia major who was receiving chelation therapy with L1 (patient 6 in Tables 1 and 2). Moderate radiographic and severe MRI changes were seen at initial study. Patient had moderate knee pain at time of initial imaging, and subsequent episodes of severe arthralgia led to discontinuation of L1 therapy. Sagittal T2-weighted RARE image (TR/TE, 3,496/100) of right knee shows joint effusion and multiple focal high-intensity lesions confined to articular cartilage (arrow). Bone marrow signal is low, which is consistent with iron deposition.

 


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Fig. 2C. 10-year-old boy with ß-thalassemia major who was receiving chelation therapy with L1 (patient 6 in Tables 1 and 2). Moderate radiographic and severe MRI changes were seen at initial study. Patient had moderate knee pain at time of initial imaging, and subsequent episodes of severe arthralgia led to discontinuation of L1 therapy. Sagittal T2-weighted RARE image (3,496/100) of right knee shows hypointense bands outlining infrapatellar fat pad (arrowheads), which is consistent with synovial hemosiderin deposition, and irregular thickening of synovial membrane (arrow).

 


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Fig. 2D. 10-year-old boy with ß-thalassemia major who was receiving chelation therapy with L1 (patient 6 in Tables 1 and 2). Moderate radiographic and severe MRI changes were seen at initial study. Patient had moderate knee pain at time of initial imaging, and subsequent episodes of severe arthralgia led to discontinuation of L1 therapy. Sagittal contrast-enhanced T1-weighted spin-echo image (624/20) obtained with fat saturation of right knee reveals intense enhancement of thickened synovium (arrows).

 


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Fig. 3A. 16-year-old boy with ß-thalassemia major 4 years after chelation therapy with L1 and 4 years after bone marrow transplantation (patient 1 in Tables 1 and 2). Severe radiographic and MRI findings were noted on follow-up imaging. Patient was asymptomatic at that time but previously had severe arthralgia during L1 treatment that resolved after discontinuation of L1. Frontal radiograph of left knee shows irregular subchondral cortical flattening of femur and tibia (arrows).

 


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Fig. 3B. 16-year-old boy with ß-thalassemia major 4 years after chelation therapy with L1 and 4 years after bone marrow transplantation (patient 1 in Tables 1 and 2). Severe radiographic and MRI findings were noted on follow-up imaging. Patient was asymptomatic at that time but previously had severe arthralgia during L1 treatment that resolved after discontinuation of L1. Lateral radiograph of left knee better reveals depth of subchondral bone lesions (arrows), which also involve patella, and shows broad beak of superior pole of patella (arrowhead).

 


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Fig. 3C. 16-year-old boy with ß-thalassemia major 4 years after chelation therapy with L1 and 4 years after bone marrow transplantation (patient 1 in Tables 1 and 2). Severe radiographic and MRI findings were noted on follow-up imaging. Patient was asymptomatic at that time but previously had severe arthralgia during L1 treatment that resolved after discontinuation of L1. Sagittal intermediate-weighted turbo spin-echo image (TR/TE, 3,099/16) of left knee reveals thickened articular cartilage extending into subchondral bone defects (arrows). Synovium (arrowheads) is mildly thickened and hypointense.

 


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Fig. 3D. 16-year-old boy with ß-thalassemia major 4 years after chelation therapy with L1 and 4 years after bone marrow transplantation (patient 1 in Tables 1 and 2). Severe radiographic and MRI findings were noted on follow-up imaging. Patient was asymptomatic at that time but previously had severe arthralgia during L1 treatment that resolved after discontinuation of L1. Axial T2-weighted turbo spin-echo image (1,912/60) obtained with fat saturation shows irregular articular surface, irregular thickness, and heterogeneous signal increase of articular cartilage (arrowheads) of right knee.

 

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