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HISTIOCYTOSIS www.freelivedoctor.com
“ Histiocytosis” <ul><li>a variety of proliferative disorders of “histiocytes” or “macrophages” </li></ul>www.freelivedoct...
Histiocyte/Dendritic Cell <ul><li>histiocyte/macrophage </li></ul><ul><li>dendritic cell (“Langerhans cell”) </li></ul>www...
Langerhans Cell  (dendritic antigen presenting cell) <ul><li>dendritic </li></ul><ul><li>antigen presenting </li></ul><ul>...
Langerhans Cell <ul><li>dendritic </li></ul><ul><li>antigen presenting </li></ul><ul><li>expresses HLA-DR and CD1a </li></...
Dendritic Cells (Reticular Cells) <ul><li>antigen-presenting cells </li></ul><ul><ul><li>1.  dendritic reticulum cells </l...
“ Histiocytosis” old classification <ul><li>Histiocytosis X </li></ul><ul><li>i.  Letterer-Siwe disease </li></ul><ul><li>...
“ Histiocytosis” classification <ul><li>Langerhans Cell Histiocytosis </li></ul><ul><ul><li>three (3) clinicopathologic en...
Acute Disseminated Langerhans Cell Histiocytosis (Letterer-Siwe disease) <ul><li>mainly occurs before 2 yrs of age </li></...
Acute Disseminated Langerhans Cell Histiocytosis (Letterer-Siwe disease) <ul><li>mainly occurs before 2 yrs of age </li></...
Acute Disseminated Langerhans Cell Histiocytosis (Letterer-Siwe disease) <ul><li>when extensive BM involvement occurs: </l...
Multifocal Langerhans Cell Histiocytosis (multiple eosinophilic granuloma) <ul><li>multiorgan involvement </li></ul><ul><u...
Multifocal Langerhans Cell Histiocytosis (multiple eosinophilic granuloma) <ul><li>Histology: </li></ul><ul><ul><li>mainly...
Multifocal Langerhans Cell Histiocytosis (multiple eosinophilic granuloma) <ul><li>Histology: </li></ul><ul><ul><li>mainly...
Multifocal Langerhans Cell Histiocytosis (multiple eosinophilic granuloma) <ul><li>Histology: </li></ul><ul><ul><li>mainly...
Unifocal Langerhans Cell Histiocytosis (unifocal eosinophilic granuloma) <ul><li>usually affects skeletal system </li></ul...
Unifocal Langerhans Cell Histiocytosis (unifocal eosinophilic granuloma) <ul><li>usually affects skeletal system </li></ul...
Histiocytic/Dendritic Cell Neoplasms (1999 WHO classification) <ul><li>Macrophage/histiocytic neoplasm </li></ul><ul><ul><...
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Histiocytosis

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Histiocytosis

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

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