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
13 - Treatment of relapsed and/or refractory 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
The last several decades have seen enormous gains in knowledge with regard to the biology of Langerhans cell histiocytosis (LCH). However, this increased understanding has not yet translated into dramatic improvements in outcome, particularly for patients with multisystem (MS) disease whose disease is refractory to standard therapy, patients with chronic relapsing disease or those with the late chronic progressive involvement of lung, liver and central nervous system (CNS), who continue to pose a significant dilemma for treating physicians. This chapter will concentrate on the problems of therapy for patients with refractory, multiorgan disease and those with late chronic progressive disease of lung and liver. CNS neurodegenerative diseases are discussed in Chapter 10.
Refractory multiorgan disease
Early cooperative group studies have shown that patients with MS disease clearly benefit from chemotherapy. The mortality for high-risk patients with disseminated disease does not, however, appear to have changed from the early Austro-German cooperative trials, the DAL-HX 83 and 90 trials, to the later LCH-I and II studies of the Histiocyte Society. Thus, about 20% of young children with MS LCH do not respond to modern protocols and often have disease that is resistant to alternative therapies.
An important step forward has been the ability to determine which patients will have poor outcomes. Features which portend a poor outcome include involvement of ‘risk’ organs (such as lung, liver, spleen and hematopoietic system) and failure to respond after 6 weeks of initial therapy (Gadner et al., 2001; Minkov et al., 2002).
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- Chapter
- Information
- Histiocytic Disorders of Children and AdultsBasic Science, Clinical Features and Therapy, pp. 254 - 271Publisher: Cambridge University PressPrint publication year: 2005
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