PMC9139267_01
Enhanced computed tomography shows thrombus formation in several arteries (yellow arrows). (A) Thrombus at the aortic arch. (B) Thrombus crosses to the left subclavian artery, leading to embolism of this artery. (C) Thrombus at the thoracic aorta. (D) Splenic infarction.
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F1
CTA of the thoracic and abdominal aorta showed thrombosis starting from the aortic arch to the left subclavian artery, accompanied by severe stenosis of the left subclavian artery, diffuse splenic infarction due to embolism of the splenic artery, and thrombosis in several arteries, including the common hepatic artery, left common iliac artery, and proximal to the femoral artery (Figure 1). Pituitary enhanced magnetic resonance imaging (MRI) revealed nodular thickening of the pituitary stalk (Supplementary Figure 1) and the desmopressin test result was positive, which were supportive of a diagnosis of diabetes insipidus.
Bone sample pathology and immunohistochemistry confirms the diagnosis of ECD. (A) Hematoxylin and eosin staining of the bone sample. (B) Foamy cells surrounded by fibrosis (black arrows). (C) CD68 staining is positive. (D) CD1a staining is negative.
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F2
For further confirmation, bone emission computed tomography (ECT), knee CT, and knee MRI were performed, which showed diffuse bone infiltration in bilateral distal femurs and proximal tibias (Supplementary Figures 2A-C). Bone puncture was finally approved, and the results showed a large number of foamy cells surrounded by fibrosis and few multinucleated giant cells (Figure 2).
Enhanced thoracic computed tomography reveals fresh thrombus formation in the brachiocephalic trunk, which regressed after interferon-alpha treatment. (A) Fresh thrombus in the brachiocephalic trunk (yellow arrow). (B) Regression of thrombus in a reviewed computed tomography after 2 months of interferon-alpha treatment.
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F3
Brain enhanced MRI revealed thickening of cerebral falx, which is a typical change in ECD (Supplementary Figure 3), while the cardiac MRI examination did not show a coated aorta. However, 1 month later, when the patient was admitted for review, enhanced CT of the thoracic aorta showed fresh thrombus formation in the brachiocephalic trunk (Figure 3A). After 2 months of treatment, repeat CT scan showed disappearance of the thrombus in the brachiocephalic trunk (Figure 3B), and her vital signs and laboratory test results returned to normal.