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Understanding Histiocytic and Dendritic Cell Disorders

Learn about diseases, neoplasms, and syndromes related to histiocytes and dendritic cells. Explore Langerhans cell histiocytosis, hemophagocytic syndrome, and more with detailed information on morphology, localization, and treatment options.

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Understanding Histiocytic and Dendritic Cell Disorders

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  1. Accesory cellsHistiocytic and dendritic cells • Ag presenting cells: dendritic • Ag processing: histiocytes • Histiocytes: phagocytic Removal of particulate Ag Derived from blood monocytes

  2. Dendritic cells In various tissues, lymph node: • Interdigitating • Follicular • Fibroblastic reticular Diseases of DC and histiocytes • Tumours of histiocytes and dendritic cells • Langerhans cell histiocytosis (/sarcoma) • Dendritic cell tumour – follicular, interdigitating, NOS

  3. Hemophagocytic syndrome • Nonneoplastic but frequently fatal proliferation of histiocytes • More common than tumours of histioc. • Complication of immunodeficiency or haematolymphoid malignancy • Often EBV-triggered

  4. Hemophagocytic syndrome • Excessive production of cytokines - cytokine storm Pancytopenia Aggressive, disseminates rapidly Responds poorly to therapy. Often fatal Localization: LN or extranodal sites Morphology: large cells: histiocytes with atypia

  5. Histiocytic and dendritic cell neoplasms • Rarest of the hematolymphoid neoplasms • Any age • Presentation and processing of Ag to lymphocytes

  6. Langerhans cell histiocytosis Synonyms: Langerhans cell granulomatosis Histiocytosis X

  7. Langerhans cell histiocytosis • Children • Adults • Incidence: 0,5-1 case /100 000 a year • Localization: bone (pain, fractures, vertebral collapse), soft tissues • Skin; lungs – adult smokers • LN, liver, BM

  8. Langerhans cell histiocytosis • Mortality - variable • Highest in children under 2 (30-50%) • Liver, lungs, BM, spleen, penetrating skull lesions

  9. Langerhans cell histiocytosis 1.unisystem, unifocal - good prognosis Adults, older chidren Bone (lytic)- skull, femur, ribs, pelvis Arrely other - lymph node, skin, lung 2. Unisystem, multifocal (Hand-Schuller-Christian) Young children, multiple destructive - bones Exophthalmos, dib. insipidus, tooth loss 3. Multifocal, multisystem (Apt-Letterer-Siwe) Infants, fever, ↑H+S, lymph nodes, pancytopenia

  10. Langerhans cell histiocytosis 4. Pulmonary Langerhans cell histiocytosis • Fibrosing interstitial disease, honeycombing, pneumothorax • Variable outcome • mortality 25% • Cessation of smoking • Corticosteroids 5. Langerhans cell sarcoma

  11. Disseminated Langerhans histiocytosis haemorrhagic eczematoid rash (10 months)

  12. Disseminated Langerhans histiocytosis osteolytic deposits in the vault

  13. Langerhans cell sarcoma

  14. Skin - hair

  15. Neoplastic Langerhans cells Imunohistochemistry CD1a + „ Coffee bean“ nuclei of LC cells and eosinophils

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