Download - histiocytosis_06-07.ppt
![Page 1: histiocytosis_06-07.ppt](https://reader036.vdocuments.net/reader036/viewer/2022070316/5560e49cd8b42a086e8b4d90/html5/thumbnails/1.jpg)
HISTIOCYTOSISHISTIOCYTOSIS
Masatoshi Kida, M.D.Dept. of Pathology
University of Vermont
![Page 2: histiocytosis_06-07.ppt](https://reader036.vdocuments.net/reader036/viewer/2022070316/5560e49cd8b42a086e8b4d90/html5/thumbnails/2.jpg)
““Histiocytosis”Histiocytosis”
a variety of proliferative disorders of “histiocytes” or “macrophages”
![Page 3: histiocytosis_06-07.ppt](https://reader036.vdocuments.net/reader036/viewer/2022070316/5560e49cd8b42a086e8b4d90/html5/thumbnails/3.jpg)
Histiocyte/Dendritic CellHistiocyte/Dendritic Cell
• histiocyte/macrophage
• dendritic cell (“Langerhans cell”)
![Page 4: histiocytosis_06-07.ppt](https://reader036.vdocuments.net/reader036/viewer/2022070316/5560e49cd8b42a086e8b4d90/html5/thumbnails/4.jpg)
Langerhans Cell Langerhans Cell (dendritic antigen presenting cell)(dendritic antigen presenting cell)
• dendritic
• antigen presenting
• expresses HLA-DR and CD1a
• cytoplasmic HX bodies (Birbeck granules)
![Page 5: histiocytosis_06-07.ppt](https://reader036.vdocuments.net/reader036/viewer/2022070316/5560e49cd8b42a086e8b4d90/html5/thumbnails/5.jpg)
Langerhans CellLangerhans Cell
• dendritic
• antigen presenting
• expresses HLA-DR and CD1a
• cytoplasmic HX bodies (Birbeck granules)
Cotran, Kumar, Collins: Pathologic Basis of Disease 1999
![Page 6: histiocytosis_06-07.ppt](https://reader036.vdocuments.net/reader036/viewer/2022070316/5560e49cd8b42a086e8b4d90/html5/thumbnails/6.jpg)
Dendritic Cells (Reticular Cells)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
![Page 7: histiocytosis_06-07.ppt](https://reader036.vdocuments.net/reader036/viewer/2022070316/5560e49cd8b42a086e8b4d90/html5/thumbnails/7.jpg)
““Histiocytosis”Histiocytosis”old classificationold classification
Histiocytosis X
i. Letterer-Siwe disease
ii. Hand-Schüller-Christian disease
iii. Eosinophilic granuloma
![Page 8: histiocytosis_06-07.ppt](https://reader036.vdocuments.net/reader036/viewer/2022070316/5560e49cd8b42a086e8b4d90/html5/thumbnails/8.jpg)
““Histiocytosis”Histiocytosis”classificationclassification
Langerhans Cell Histiocytosis
three (3) clinicopathologic entities1. acute disseminated Langerhans cell hitiocytosis (Letterer-Siwe disease)
2. multifocal Langerhans cell histiocytosis (multifocal eosinophilic granuloma)
3. unifocal Langerhans cell histiocytosis (unifocal eosinophilic granuloma)
![Page 9: histiocytosis_06-07.ppt](https://reader036.vdocuments.net/reader036/viewer/2022070316/5560e49cd8b42a086e8b4d90/html5/thumbnails/9.jpg)
Acute Disseminated Langerhans Cell Acute Disseminated Langerhans Cell Histiocytosis (Letterer-Siwe disease)Histiocytosis (Letterer-Siwe disease)
• mainly occurs before 2 yrs of age
• skin involvement (trunk, scalp)
• hepatosplenomegaly, lymphadenopathy, lung involvement
• destructive osteolytic bone lesions
![Page 10: histiocytosis_06-07.ppt](https://reader036.vdocuments.net/reader036/viewer/2022070316/5560e49cd8b42a086e8b4d90/html5/thumbnails/10.jpg)
Acute Disseminated Langerhans Cell Acute Disseminated Langerhans Cell Histiocytosis (Letterer-Siwe disease)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
![Page 11: histiocytosis_06-07.ppt](https://reader036.vdocuments.net/reader036/viewer/2022070316/5560e49cd8b42a086e8b4d90/html5/thumbnails/11.jpg)
Acute Disseminated Langerhans Cell Acute Disseminated Langerhans Cell Histiocytosis (Letterer-Siwe disease)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
![Page 12: histiocytosis_06-07.ppt](https://reader036.vdocuments.net/reader036/viewer/2022070316/5560e49cd8b42a086e8b4d90/html5/thumbnails/12.jpg)
Multifocal Langerhans Cell Histiocytosis Multifocal Langerhans Cell Histiocytosis (multiple eosinophilic granuloma)(multiple eosinophilic granuloma)
• multiorgan involvementbone (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
![Page 13: histiocytosis_06-07.ppt](https://reader036.vdocuments.net/reader036/viewer/2022070316/5560e49cd8b42a086e8b4d90/html5/thumbnails/13.jpg)
Multifocal Langerhans Cell Histiocytosis Multifocal Langerhans Cell Histiocytosis (multiple eosinophilic granuloma)(multiple eosinophilic granuloma)
Histology:• mainly involving medullary cavity of bone• expanding, erosive accumulation of Langerhans cells
![Page 14: histiocytosis_06-07.ppt](https://reader036.vdocuments.net/reader036/viewer/2022070316/5560e49cd8b42a086e8b4d90/html5/thumbnails/14.jpg)
Multifocal Langerhans Cell Histiocytosis Multifocal Langerhans Cell Histiocytosis (multiple eosinophilic granuloma)(multiple eosinophilic granuloma)
Histology:• mainly involving medullary cavity of bone• expanding, erosive accumulation of Langerhans cells• variably admixed with eosinophils, lymphocytes, plasma
cells, neutrophils
![Page 15: histiocytosis_06-07.ppt](https://reader036.vdocuments.net/reader036/viewer/2022070316/5560e49cd8b42a086e8b4d90/html5/thumbnails/15.jpg)
Multifocal Langerhans Cell Histiocytosis Multifocal Langerhans Cell Histiocytosis (multiple eosinophilic granuloma)(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
![Page 16: histiocytosis_06-07.ppt](https://reader036.vdocuments.net/reader036/viewer/2022070316/5560e49cd8b42a086e8b4d90/html5/thumbnails/16.jpg)
Unifocal Langerhans Cell Histiocytosis Unifocal Langerhans Cell Histiocytosis (unifocal eosinophilic granuloma)(unifocal eosinophilic granuloma)
• usually affects skeletal system• may be asymptomatic or may cause pain and tenderness• an indolent disease
![Page 17: histiocytosis_06-07.ppt](https://reader036.vdocuments.net/reader036/viewer/2022070316/5560e49cd8b42a086e8b4d90/html5/thumbnails/17.jpg)
Unifocal Langerhans Cell Histiocytosis Unifocal Langerhans Cell Histiocytosis (unifocal eosinophilic granuloma)(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
![Page 18: histiocytosis_06-07.ppt](https://reader036.vdocuments.net/reader036/viewer/2022070316/5560e49cd8b42a086e8b4d90/html5/thumbnails/18.jpg)
Histiocytic/Dendritic Cell Neoplasms Histiocytic/Dendritic Cell Neoplasms (1999 WHO classification)(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
![Page 19: histiocytosis_06-07.ppt](https://reader036.vdocuments.net/reader036/viewer/2022070316/5560e49cd8b42a086e8b4d90/html5/thumbnails/19.jpg)
![Page 20: histiocytosis_06-07.ppt](https://reader036.vdocuments.net/reader036/viewer/2022070316/5560e49cd8b42a086e8b4d90/html5/thumbnails/20.jpg)
![Page 21: histiocytosis_06-07.ppt](https://reader036.vdocuments.net/reader036/viewer/2022070316/5560e49cd8b42a086e8b4d90/html5/thumbnails/21.jpg)
![Page 22: histiocytosis_06-07.ppt](https://reader036.vdocuments.net/reader036/viewer/2022070316/5560e49cd8b42a086e8b4d90/html5/thumbnails/22.jpg)
![Page 23: histiocytosis_06-07.ppt](https://reader036.vdocuments.net/reader036/viewer/2022070316/5560e49cd8b42a086e8b4d90/html5/thumbnails/23.jpg)
![Page 24: histiocytosis_06-07.ppt](https://reader036.vdocuments.net/reader036/viewer/2022070316/5560e49cd8b42a086e8b4d90/html5/thumbnails/24.jpg)
![Page 25: histiocytosis_06-07.ppt](https://reader036.vdocuments.net/reader036/viewer/2022070316/5560e49cd8b42a086e8b4d90/html5/thumbnails/25.jpg)
![Page 26: histiocytosis_06-07.ppt](https://reader036.vdocuments.net/reader036/viewer/2022070316/5560e49cd8b42a086e8b4d90/html5/thumbnails/26.jpg)
![Page 27: histiocytosis_06-07.ppt](https://reader036.vdocuments.net/reader036/viewer/2022070316/5560e49cd8b42a086e8b4d90/html5/thumbnails/27.jpg)
![Page 28: histiocytosis_06-07.ppt](https://reader036.vdocuments.net/reader036/viewer/2022070316/5560e49cd8b42a086e8b4d90/html5/thumbnails/28.jpg)
![Page 29: histiocytosis_06-07.ppt](https://reader036.vdocuments.net/reader036/viewer/2022070316/5560e49cd8b42a086e8b4d90/html5/thumbnails/29.jpg)
![Page 30: histiocytosis_06-07.ppt](https://reader036.vdocuments.net/reader036/viewer/2022070316/5560e49cd8b42a086e8b4d90/html5/thumbnails/30.jpg)
![Page 31: histiocytosis_06-07.ppt](https://reader036.vdocuments.net/reader036/viewer/2022070316/5560e49cd8b42a086e8b4d90/html5/thumbnails/31.jpg)