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
6 - Langerhans cell histiocytosis: a clinical update
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
In 1953, Lichtenstein coined the term histiocytosis X for the different forms of Langerhans cell histiocytosis (LCH), the X denoting his uncertainty about the disease and its pathogenesis (Lichtenstein, 1953). Fifty years later some of the uncertainty still exists. The histopathological appearance of the various forms of ‘LCH’ is identical, yet the intriguing question remains. How is it that patients with such a variable clinical profile, from a single self-healing bone lesion to a generalized disorder resistant to any known therapy, seem to have the same disease? Many more pieces of the ‘LCH jigsaw puzzle’ are still missing and need to be fitted in. Mean while, descriptions of the clinical features, the natural history of the disease and the long-term outcome provide a useful framework for clinicians, for recognition and management of the disease. In describing the clinical picture of LCH, we have focused on those few studies which have included more than 50 patients (Table 6.1). It is noteworthy that until April 2004, only 14 studies, the product of just 10 centres or cooperative groups, have reported on more than 100 patients.
Clinical involvement by specific organs
The frequency of involvement of specific organs or ‘organ systems’ is described in the following sections in descending order of frequency. Most cooperative studies record specific system involvement at diagnosis; in some cases, events during the course of LCH are also included.
Bone
In most surveys, bone lesions are present in around 80% of patients.
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- Histiocytic Disorders of Children and AdultsBasic Science, Clinical Features and Therapy, pp. 95 - 129Publisher: Cambridge University PressPrint publication year: 2005
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