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Accesory cells Histiocytic and dendritic cells

Accesory cells Histiocytic and dendritic cells. Ag presenting cells: dendritic Ag processing: histiocytes Histiocytes: phagocytic Removal of particulate Ag Derived from blood monocytes. Dendritic cells. In various tissues, lymph node: Interdigitating Follicular

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Accesory cells Histiocytic and dendritic cells

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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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