histiocytosis_06-07.ppt

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histiocytosis_06-07.ppt

  1. 1. HISTIOCYTOSIS Masatoshi Kida, M.D. Dept. of Pathology University of Vermont
  2. 2. “Histiocytosis” a variety of proliferative disorders of “histiocytes” or “macrophages”
  3. 3. Histiocyte/Dendritic Cell • histiocyte/macrophage • dendritic cell (“Langerhans cell”)
  4. 4. Langerhans Cell (dendritic antigen presenting cell) • dendritic • antigen presenting • expresses HLA-DR and CD1a • cytoplasmic HX bodies (Birbeck granules)
  5. 5. Langerhans Cell • dendritic • antigen presenting • expresses HLA-DR and CD1a • cytoplasmic HX bodies (Birbeck granules) Cotran, Kumar, Collins: Pathologic Basis of Disease 1999
  6. 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
  7. 7. “Histiocytosis” old classification Histiocytosis X i. Letterer-Siwe disease ii. Hand-Schüller-Christian disease iii. Eosinophilic granuloma
  8. 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)
  9. 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
  10. 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
  11. 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
  12. 12. Multifocal Langerhans Cell Histiocytosis (multiple eosinophilic granuloma) • multiorgan involvement bone (calvaria, ribs, femur),skin, lungs, stomach • mainly occurs in children • fever, diffuseHand-Schuller-Christian triad eruptions (scalp, ear canals) • frequent bouts of otitis media, mastoiditis, upper respiratory tract infections 1. Calvarial defects • lymphadenopathy, hepatomegaly, splenomegaly 2. Diabetes insipidus • diabetes insipidus <--- post pituitary stalk involvement (~50% of cases) 3. Exophthalmos • “Hand-Schüller-Christian triad”
  13. 13. Multifocal Langerhans Cell Histiocytosis (multiple eosinophilic granuloma) Histology: • mainly involving medullary cavity of bone • expanding, erosive accumulation of Langerhans cells
  14. 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
  15. 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
  16. 16. Unifocal Langerhans Cell Histiocytosis (unifocal eosinophilic granuloma) • usually affects skeletal system • may be asymptomatic or may cause pain and tenderness • an indolent disease
  17. 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
  18. 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

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