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Histiocytosis
Histiocytosis
Histiocytosis
Histiocytosis
Histiocytosis
Histiocytosis
Histiocytosis
Histiocytosis
Histiocytosis
Histiocytosis
Histiocytosis
Histiocytosis
Histiocytosis
Histiocytosis
Histiocytosis
Histiocytosis
Histiocytosis
Histiocytosis
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Histiocytosis

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  • 1. HISTIOCYTOSIS www.freelivedoctor.com
  • 2. “ Histiocytosis”
    • a variety of proliferative disorders of “histiocytes” or “macrophages”
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  • 3. Histiocyte/Dendritic Cell
    • histiocyte/macrophage
    • dendritic cell (“Langerhans cell”)
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  • 4. Langerhans Cell (dendritic antigen presenting cell)
    • dendritic
    • antigen presenting
    • expresses HLA-DR and CD1a
    • cytoplasmic HX bodies (Birbeck granules)
    www.freelivedoctor.com
  • 5. Langerhans Cell
    • dendritic
    • antigen presenting
    • expresses HLA-DR and CD1a
    • cytoplasmic HX bodies (Birbeck granules)
    Cotran, Kumar, Collins: Pathologic Basis of Disease 1999 www.freelivedoctor.com
  • 6. Dendritic Cells (Reticular Cells)
    • antigen-presenting cells
      • 1. dendritic reticulum cells
      • (dendritic follicular cells)
      • 2. interdigitating reticulum cells
      • (dendritic interdigitating cells, dendritic cells)
      • 3. fibroblastic reticulum cells
      • 4. histiocytic reticulum cells
      • 5. other “reticulum” cells
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  • 7. “ Histiocytosis” old classification
    • Histiocytosis X
    • i. Letterer-Siwe disease
    • ii. Hand-Schüller-Christian disease
    • iii. Eosinophilic granuloma
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  • 8. “ Histiocytosis” classification
    • Langerhans Cell Histiocytosis
      • three (3) clinicopathologic entities
        • 1. acute disseminated Langerhans cell hitiocytosis
        • (Letterer-Siwe disease)
        • 2. multifocal Langerhans cell histiocytosis
        • (multifocal eosinophilic granuloma)
        • 3. unifocal Langerhans cell histiocytosis
        • (unifocal eosinophilic granuloma)
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  • 9. Acute Disseminated Langerhans Cell Histiocytosis (Letterer-Siwe disease)
    • mainly occurs before 2 yrs of age
    • skin involvement (trunk, scalp)
    • hepatosplenomegaly, lymphadenopathy, lung involvement
    • destructive osteolytic bone lesions
    www.freelivedoctor.com
  • 10. Acute Disseminated Langerhans Cell Histiocytosis (Letterer-Siwe disease)
    • mainly occurs before 2 yrs of age
    • skin involvement (trunk, scalp)
    • hepatosplenomegaly, lymphadenopathy, lung involvement
    • destructive osteolytic bone lesions
    Cotran, Kumar, Collins: Pathologic Basis of Disease 1999 www.freelivedoctor.com
  • 11. Acute Disseminated Langerhans Cell Histiocytosis (Letterer-Siwe disease)
    • when extensive BM involvement occurs:
      • anemia, thrombocytopenia,
      • recurrent infection (otitis media, mastoiditis)
    • if untreated ---> rapidly fatal
    • with intensive chemotherapy
    • ---> 50% 5-yr-survival
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  • 12. Multifocal Langerhans Cell Histiocytosis (multiple eosinophilic granuloma)
    • multiorgan involvement
      • bone ( calvaria , ribs, femur),skin, lungs, stomach
    • mainly occurs in children
    • fever, diffuse eruptions (scalp, ear canals)
    • frequent bouts of otitis media, mastoiditis, upper respiratory tract infections
    • lymphadenopathy, hepatomegaly, splenomegaly
    • diabetes insipidus <--- post pituitary stalk involvement (~50% of cases)
    • “ Hand-Schüller-Christian triad”
    Hand-Schuller-Christian triad 1. Calvarial defects 2. Diabetes insipidus 3. Exophthalmos www.freelivedoctor.com
  • 13. Multifocal Langerhans Cell Histiocytosis (multiple eosinophilic granuloma)
    • Histology:
      • mainly involving medullary cavity of bone
      • expanding, erosive accumulation of Langerhans cells
    www.freelivedoctor.com
  • 14. Multifocal Langerhans Cell Histiocytosis (multiple eosinophilic granuloma)
    • Histology:
      • mainly involving medullary cavity of bone
      • expanding, erosive accumulation of Langerhans cells
      • variably admixed with eosinophils, lymphocytes, plasma cells, neutrophils
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  • 15. Multifocal Langerhans Cell Histiocytosis (multiple eosinophilic granuloma)
    • Histology:
      • mainly involving medullary cavity of bone
      • expanding, erosive accumulation of Langerhans cells
      • variably admixed with eosinophils, lymphocytes, plasma cells, neutrophils
    • Treatment:
      • 1. may undergo spontaneous regression
      • 2. chemotherapy
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  • 16. Unifocal Langerhans Cell Histiocytosis (unifocal eosinophilic granuloma)
    • usually affects skeletal system
    • may be asymptomatic or may cause pain and tenderness
    • an indolent disease
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  • 17. Unifocal Langerhans Cell Histiocytosis (unifocal eosinophilic granuloma)
    • usually affects skeletal system
    • may be asymptomatic or may cause pain and tenderness
    • an indolent disease
    • Treatment:
      • 1. may undergo spontaneous regression
      • 2. local excision
      • 3. local irradiation
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  • 18. Histiocytic/Dendritic Cell Neoplasms (1999 WHO classification)
    • Macrophage/histiocytic neoplasm
      • Histiocytic sarcoma
    • Dendritic cell neoplasms
      • Langerhans cell histiocytosis
      • Langerhans cell sarcoma
      • Interdigitating dendritic cell sarcoma/tumor
      • Follicular dendritic cell sarcoma/tumor
      • Dendritic cell sarcoma, NOS
    www.freelivedoctor.com

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