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
12 - The treatment of Langerhans cell 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
Background
The treatment of Langerhans cell histiocytosis (LCH) has varied greatly over the past century, and is still controversial. Early treatment approaches reflected contemporary views on disease pathogenesis, which included granulomatous, inflammatory or infectious origins for LCH. Consequently, children with LCH were treated with antibiotics, anti-inflammatory agents including steroids, and with radiation therapy and over the last 30–40 years with cytotoxic chemotherapy. While varying degrees of success have been reported, it remains true that only once the issues of aetiology and pathogenesis have been resolved can a definitive therapy be envisioned. Nevertheless, systematic approaches to diagnosis and treatment, which were major advances of the 1980s, have improved the outlook for children with LCH and are the main focus of this chapter.
Historical perspective
The first systematic treatment trial of children with LCH was that of Lahey (1962). In that study, children were matched for age and extent of disease, and outcome was analysed according to whether or not ‘specific’ treatment for LCH was given. Treatment was not controlled however, and a variety of agents, including antibiotics, steroids and cytotoxic drugs were given, making direct comparisons problematic. Nevertheless, the principal, and important, finding of this study was a significant increase in survival in the group of children receiving therapy compared to those who were untreated.
These findings led to a number of studies in which, unfortunately, the patient populations varied greatly with respect to extent of disease. This variability made interpretation of results difficult, and conclusions regarding superior treatment approaches were at best tenuous.
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- Chapter
- Information
- Histiocytic Disorders of Children and AdultsBasic Science, Clinical Features and Therapy, pp. 229 - 253Publisher: Cambridge University PressPrint publication year: 2005
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