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
9 - Special aspects of Langerhans cell histiocytosis in the adult
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) may affect patients of any age, from the newborn to the elderly (Nezelof et al., 1979; Aricò and Egeler, 1998; Aricò et al., 2003). For several reasons this disease has been more familiar to pediatricians than to specialists of adult patients, and in adults the features of this disease are still poorly defined. Thus, most of the available information concerning clinical features, pathogenesis, and treatment outcome derives from the pediatric experience. Several features of the disease are common to pediatric and adult patients who may both have either localized or disseminated disease. The proportion of cases with lung involvement is much higher in adults, however. This can be partly explained by cigarette smoking, but other differences between pediatric and adult LCH still require elucidation.
Background and clinical features
Only a limited number of literature reports are available which describe series of patients with LCH diagnosed during adulthood, and only a few describe relatively large series of patients. Furthermore, most of these reports derive from single-specialty experiences, which may carry an inherent selection bias. In addition, continued uncertainty with regard to the pathogenesis of adult LCH has limited our current ability to devise optimal therapy, and no randomized clinical trials have been conducted in adults so far. Thus, most information derives from the descriptions of single or a few cases, often reported retrospectively. Forty years ago, Lewis reported 12 patients with ‘eosinophilic granuloma and its variants with special reference to lung involvement’ (Lewis, 1964).
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- Information
- Histiocytic Disorders of Children and AdultsBasic Science, Clinical Features and Therapy, pp. 174 - 186Publisher: Cambridge University PressPrint publication year: 2005
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