The clinical spectrum of Erdheim-Chester disease: an observational cohort study by Juvianee I. Estrada-Veras, Kevin J. O’Brien, Louisa C. Boyd, Rahul H.

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Presentation transcript:

The clinical spectrum of Erdheim-Chester disease: an observational cohort study by Juvianee I. Estrada-Veras, Kevin J. O’Brien, Louisa C. Boyd, Rahul H. Dave, Benjamin H. Durham, Liqiang Xi, Ashkan A. Malayeri, Marcus Y. Chen, Pamela J. Gardner, Jhonell R. Alvarado Enriquez, Nikeith Shah, Omar Abdel-Wahab, Bernadette R. Gochuico, Mark Raffeld, Elaine S. Jaffe, and William A. Gahl BloodAdv Volume 1(6):357-366 February 14, 2017 ©2017 by American Society of Hematology

Juvianee I. Estrada-Veras et al. Blood Adv 2017;1:357-366 ©2017 by American Society of Hematology

Bone lesions in ECD. The red arrows show: (A) Whole-body bone scan with technetium-99 showing avid uptake in the knees and left hip of an ECD patient. Bone lesions in ECD. The red arrows show: (A) Whole-body bone scan with technetium-99 showing avid uptake in the knees and left hip of an ECD patient. Less intense uptake occurs bilaterally in the humeri and distal tibias. (B) FDG PET-CT scan showing increased FDG uptake in the knees and proximal and distal tibias. (C) Bone radiograph showing cortical osteosclerosis in the right humeri. (D) Bone radiograph showing cortical osteosclerosis in the distal right radius. (E) CT scan of the right knee of an ECD patient showing cortical osteosclerosis and mottled appearance of the bone. (F) MRI scan of the right knee of an ECD patient showing serpiginous areas of T1 signal hypointensity, indicative of osteosclerosis. (G) Panoramic radiograph showing bilateral osteosclerosis of the mandible and maxillary sinus disease. Juvianee I. Estrada-Veras et al. Blood Adv 2017;1:357-366 ©2017 by American Society of Hematology

Cardiovascular, retroperitoneal, and lung images of ECD patients. Cardiovascular, retroperitoneal, and lung images of ECD patients. (A) Sagittal reconstruction postcontrast CT demonstrates encasement of the thoracic aorta down to the bifurcation. (B) Coronal FDG PET-CT scan showing increased FDG uptake in the thoracic and abdominal aorta. Symmetrically encased kidneys “hairy kidney” showing increased FDG uptake. (C) Axial CT with contrast demonstrates mass-like enhancement encasing the kidneys symmetrically (“hairy kidney”). In addition, there is circumferential encasement and narrowing of the abdominal aorta (arrow). (D) Cardiac CT showing partial encasement of the right coronary artery. (E) Cardiac CT showing partial encasement of the right coronary artery. (F) Axial postcontrast CT image of the upper abdomen demonstrating mass-like perinephric stranding surrounding the kidneys with bilateral hydronephrosis. Hyperdense material within the right collecting system is a ureteral stent. (G) Postcontrast coronal MRI image of the kidneys demonstrating extension of the perinephric mass into the adrenal bed and encasement of the adrenals (arrows). (H) High-resolution CT of the chest showing interstitial fibrosis. Juvianee I. Estrada-Veras et al. Blood Adv 2017;1:357-366 ©2017 by American Society of Hematology

Brain images in ECD. (A) Axial post contrast brain MRI showing suprasellar and cerebellar involvement in a patient with ECD. (B) Sagittal post contrast brain MRI (from panel A) showing suprasellar and cerebellar tumors in a patient with ECD. (C) Axial fluid attenuated inversion recovery brain MRI showing ECD tumors in cerebral hemispheres. Brain images in ECD. (A) Axial post contrast brain MRI showing suprasellar and cerebellar involvement in a patient with ECD. (B) Sagittal post contrast brain MRI (from panel A) showing suprasellar and cerebellar tumors in a patient with ECD. (C) Axial fluid attenuated inversion recovery brain MRI showing ECD tumors in cerebral hemispheres. (D) Axial fluid attenuated inversion recovery brain MRI showing ECD tumors in cerebral hemisphere. (E) Sagittal post contrast brain MRI showing an ECD tumor with cystic components in the midbrain-pons of a patient with ECD. (F) T2 fluid attenuated inversion recovery MRI image showing increased symmetrical signal intensity in the cerebellum. (G) Neurodegeneration and atrophy of the cerebellum in a patient with ECD seen on brain MRI. (H) Orbital involvement with tissue accumulation in the intraconal space secondary to histiocytes accumulation in ECD. (I) Orbital involvement showing increased thickening of the lateral rectus muscle. (J) Pituitary stalk is thickened secondary to macrophage accumulation in ECD and deviated to the right. (K) Hematoxylin and eosin stain for brain lesion showing foamy macrophages and inflammation in brain mass biopsy specimen (original magnification ×40). (L) CD68 KP-1 stain of panel K highlighting the foamy macrophages (original magnification ×40). Juvianee I. Estrada-Veras et al. Blood Adv 2017;1:357-366 ©2017 by American Society of Hematology

Skin findings in ECD. (A) Periorbital xanthelasmas in a patient with ECD. Note mild exophthalmos secondary to retro-orbital mass. Skin findings in ECD. (A) Periorbital xanthelasmas in a patient with ECD. Note mild exophthalmos secondary to retro-orbital mass. (B) Upper lid xanthelasmas are apparent when the patient’s eyes are closed. (C) Skin lesions containing foamy macrophages negative for S-100 protein but with activating ALK gene fusion. (D) Skin lesion positive for BRAF V600E mutation. (E) Foamy macrophages in skin lesion. Inset shows lipid-laden macrophages interspersed with inflammatory cells (original magnification ×20; hematoxylin and eosin stain). Juvianee I. Estrada-Veras et al. Blood Adv 2017;1:357-366 ©2017 by American Society of Hematology