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
14 - Late effects of Langerhans cell histiocytosis and its association with malignancy
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), although considered a benign and treatable condition, can result in sequelae in the various tissues involved. Some of the problems, such as diabetes insipidus (DI), can arise at the time of diagnosis of LCH or even before. It has therefore been suggested that the term ‘permanent consequences’ might be better than the term ‘late effects’ when describing these disabilities (Gadner et al., 1994).
It was recognized decades ago that up to half the survivors of LCH may have residual disabilities which impact on the quality of survival (Komp et al., 1980; Komp, 1980). There have since been reports from single institutions and from co-operative national groups describing long-term complications in LCH patients (Sims, 1977; Ceci et al., 1993; Gadner et al., 1994; French LCH Study Group, 1996; Willis et al., 1996; Haupt et al., 2004). However, there are considerable differences in the reported incidence and prevalence of sequelae, ranging from 20% to 70%, in the various studies. This discrepancy may be due to several different factors including the study size, selection of patient cohorts, treatments used, definitions for diagnosis of sequelae, referral bias to institutions and methods used for follow-up assessment (telephone interviews, questionnaire-based studies and clinical examination) (Table 14.1).
Most of the published literature on permanent consequences are follow-up studies in subjects who had LCH during childhood.
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- Histiocytic Disorders of Children and AdultsBasic Science, Clinical Features and Therapy, pp. 272 - 292Publisher: Cambridge University PressPrint publication year: 2005
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