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

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Oct 29, 2022 328 likes •526 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

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Erdheim-Chester Disease

A clonal histiocytic neoplasm affecting multiple organ systems ELAINE S. JAFFE, MD National Cancer Institute

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28% 94% 30% 20% 44%

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75 year old male Bone pain, fatigue Increased uptake in femur, tibia CT of abdomen shows perirenal stranding

Biopsy, Left Distal Femur

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Osteosclerosis

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Fibrosis, Histiocytic infiltrate

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CD68 CD163

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Positive for BRAF V600E mutation Bone biopsies must be processed in EDTA – not formic acid for decalcification

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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

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Touton Giant cells

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CD68

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45 year old male with a sclerotic lesion of the femur

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Fibrosis

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Foamy histiocytes

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Foamy histiocytes, giant cells Lymphocytes and plasma cells

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