Book contents
- Frontmatter
- Contents
- List of contributors
- Preface
- 1 Histiocytic disorders of children and adults: introduction to the problem, overview, historical perspective and epidemiology
- 2 The diagnostic histopathology of Langerhans cell histiocytosis
- 3 Histiocyte function and development in the normal immune system
- 4 The immunological basis of Langerhans cell histiocytosis
- 5 The genetics of Langerhans cell histiocytosis
- 6 Langerhans cell histiocytosis: a clinical update
- 7 Histiocytosis of the skin in children and adults
- 8 Langerhans cell histiocytosis of bone
- 9 Special aspects of Langerhans cell histiocytosis in the adult
- 10 Adult lung histiocytosis
- 11 Central nervous system disease in Langerhans cell histiocytosis
- 12 The treatment of Langerhans cell histiocytosis
- 13 Treatment of relapsed and/or refractory Langerhans cell histiocytosis
- 14 Late effects of Langerhans cell histiocytosis and its association with malignancy
- 15 Uncommon histiocytic disorder: the non-Langerhans cell histiocytoses
- 16 The histopathology of hemophagocytic lymphohistiocytosis
- 17 Genetics and pathogenesis of hemophagocytic lymphohistiocytosis
- 18 Clinical aspects and therapy of hemophagocytic lymphohistiocytosis
- 19 Secondary haemophagocytic syndromes associated with rheumatic diseases
- 20 Malignancies of the monocyte/macrophage system
- 21 Psychosocial aspects of the histiocytic disorders: staying on course under challenging clinical circumstances
- Index
- Plate section
10 - Adult lung histiocytosis
Published online by Cambridge University Press: 27 August 2009
- Frontmatter
- Contents
- List of contributors
- Preface
- 1 Histiocytic disorders of children and adults: introduction to the problem, overview, historical perspective and epidemiology
- 2 The diagnostic histopathology of Langerhans cell histiocytosis
- 3 Histiocyte function and development in the normal immune system
- 4 The immunological basis of Langerhans cell histiocytosis
- 5 The genetics of Langerhans cell histiocytosis
- 6 Langerhans cell histiocytosis: a clinical update
- 7 Histiocytosis of the skin in children and adults
- 8 Langerhans cell histiocytosis of bone
- 9 Special aspects of Langerhans cell histiocytosis in the adult
- 10 Adult lung histiocytosis
- 11 Central nervous system disease in Langerhans cell histiocytosis
- 12 The treatment of Langerhans cell histiocytosis
- 13 Treatment of relapsed and/or refractory Langerhans cell histiocytosis
- 14 Late effects of Langerhans cell histiocytosis and its association with malignancy
- 15 Uncommon histiocytic disorder: the non-Langerhans cell histiocytoses
- 16 The histopathology of hemophagocytic lymphohistiocytosis
- 17 Genetics and pathogenesis of hemophagocytic lymphohistiocytosis
- 18 Clinical aspects and therapy of hemophagocytic lymphohistiocytosis
- 19 Secondary haemophagocytic syndromes associated with rheumatic diseases
- 20 Malignancies of the monocyte/macrophage system
- 21 Psychosocial aspects of the histiocytic disorders: staying on course under challenging clinical circumstances
- Index
- Plate section
Summary
Introduction
Langerhans cell histiocytosis (LCH) encompasses a group of disorders of unknown origin with diverse clinical presentations and outcomes, characterized by infiltration of involved tissues by pathologic Langerhans cells (LCs). Acute disseminated LCH is a severe multisystem disease that predominantly affects young children. Multifocal LCH is seen mainly in older children and adolescents and runs a variable but usually more favorable course. Single-system disease (eosinophilic granuloma) is characterized by involvement of a single organ (bone, lungs, or skin) and often follows a benign course (Howarth et al., 1999). Pulmonary involvement in patients with multisystem disease is rarely at the forefront of the clinical picture. Isolated or predominant pulmonary involvement is the pattern encountered by pulmonologists in adults and has a number of specific epidemiological and clinical features that warrant its individualization as a separate entity (Tazi et al., 2000; Vassallo et al., 2000; Sundar et al., 2003).
Epidemiology
Adult lung LCH is an uncommon disorder that occurs almost exclusively in smokers. In an early series of more than 500 patients with diffuse infiltrating lung disease, surgical lung biopsy showed LCH in less than 5% of cases (Gaensler and Carrington, 1980). Several studies have documented comparable data thereafter as reviewed in Tazi et al. (2000) and Vassallo et al. (2000). The prevalence of pulmonary LCH (pLCH) is, however, probably underestimated because some patients are asymptomatic or experience spontaneous remission, and histological findings are nonspecific in advanced forms. The wide use of chest high-resolution computed tomography (HRCT) in the evaluation of patients may increase the number of patients in whom pLCH is diagnosed.
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- Information
- Histiocytic Disorders of Children and AdultsBasic Science, Clinical Features and Therapy, pp. 187 - 207Publisher: Cambridge University PressPrint publication year: 2005
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