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Histiocytoses

8

 

Paul Imbach

 

Contents

8.1

Definition and Overview ..................................................................................................

75

8.2

Langerhans Cell Histiocytosis .........................................................................................

76

 

8.2.1

Incidence ..............................................................................................................

76

 

8.2.2

Etiology and Pathogenesis ...................................................................................

76

 

8.2.3

Histopathology.....................................................................................................

77

 

8.2.4

Clinical Presentation ............................................................................................

81

 

8.2.5

Differential Diagnosis ..........................................................................................

81

 

8.2.6

Prognosis..............................................................................................................

81

 

8.2.7

General Therapeutic Approach ............................................................................

81

 

8.2.8

Long-Term Sequelae............................................................................................

82

 

8.2.9 Special Clinical Presentations of (LCH)..............................................................

83

8.3 Infection-Associated Hemophagocytic Syndrome (IAHS)..............................................

84

8.4 Familial Erythrophagocytic Lymphohistiocytosis (FEL).................................................

84

 

8.4.1

Definition .............................................................................................................

84

 

8.4.2

Pathology and Genetics........................................................................................

85

 

8.4.3

Clinical Presentation ............................................................................................

85

 

8.4.4

Laboratory Analyses ............................................................................................

85

 

8.4.5

Clinical Course.....................................................................................................

86

 

8.4.6

Differential Diagnosis ..........................................................................................

86

 

8.4.7

Therapy ................................................................................................................

86

8.5

Malignant Histiocytosis ...................................................................................................

86

 

8.5.1

Incidence ..............................................................................................................

86

 

8.5.2

Pathology .............................................................................................................

86

 

8.5.3

Clinical Presentation ............................................................................................

87

 

8.5.4

Therapy ................................................................................................................

87

8.1Definition and Overview

Histiocytoses are characterized by histiocytic infiltration of tissue and organ systems in localized or disseminated forms.

P. Imbach et al. (eds.), Pediatric Oncology,

75

DOI 10.1007/978-3-642-20359-6_8, © Springer-Verlag Berlin Heidelberg 2011

 

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