Erdheim-Chester Disease A clonal histiocytic neoplasm affecting - PowerPoint PPT Presentation

Oct 29, 2022 •328 likes •520 views

Erdheim-Chester Disease A clonal histiocytic neoplasm affecting multiple organ systems ELAINE S. JAFFE, MD National Cancer Institute 30% 28% 44% 20% 94% 75 year old male Bone pain, fatigue Increased uptake in femur, tibia CT of abdomen

Erdheim-Chester Disease A clonal histiocytic neoplasm affecting multiple organ systems ELAINE S. JAFFE, MD National Cancer Institute
30% 28% 44% 20% 94%
75 year old male Bone pain, fatigue Increased uptake in femur, tibia CT of abdomen shows perirenal stranding Biopsy, Left Distal Femur
Osteosclerosis
Fibrosis, Histiocytic infiltrate
CD68 CD163
Positive for BRAF V600E mutation Bone biopsies must be processed in EDTA – not formic acid for decalcification
53 year old female Progressive headache, loss of balance MRI showed cerebellar lesion and muliple lesions surrounding fourth ventricle, pons, left occipital lobe, right parietal lobe Biopsy of cerebellum
Touton Giant cells
CD68
45 year old male with a sclerotic lesion of the femur
Fibrosis
Foamy histiocytes
Foamy histiocytes, giant cells Lymphocytes and plasma cells
CD163 CD68

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