Histiocytosis
Transcript of Histiocytosis
HISTIOCYTOSISHISTIOCYTOSIS
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““Histiocytosis”Histiocytosis”
a variety of proliferative disorders of “histiocytes” or “macrophages”
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Histiocyte/Dendritic CellHistiocyte/Dendritic Cell
• histiocyte/macrophage
• dendritic cell (“Langerhans cell”)
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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)
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Langerhans CellLangerhans Cell
• dendritic
• antigen presenting
• expresses HLA-DR and CD1a
• cytoplasmic HX bodies (Birbeck granules)
Cotran, Kumar, Collins: Pathologic Basis of Disease 1999www.freelivedoctor.com
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
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““Histiocytosis”Histiocytosis”old classificationold classification
Histiocytosis X
i. Letterer-Siwe disease
ii. Hand-Schüller-Christian disease
iii. Eosinophilic granuloma
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““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)
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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
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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
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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
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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
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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
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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
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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
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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
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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
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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
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