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
8 - Langerhans cell histiocytosis of bone
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
Bone is the commonest single organ involved in Langerhans cell histiocytosis (LCH) in children. Of 154 consecutive children admitted to a single large institution, 126 (81%) had bone involvement, of whom 80% had single system (SS) disease and 20% bone disease as part of multisystem (MS) LCH. A single bone was involved in 76% – unifocal bone (UFB) and two or more bones – multifocal bone (MFB) in 24% (Stuurman et al., 2003), correlating almost exactly with the 78% UFB and 22% MFB found by the DAL-HX group (Titgemeyer et al., 2001).
The commonest presentation of LCH in children is thus with UFB. The picture is different in adults in whom SS skin and lung disease predominate, and in whom bone is more often part of MS disease (Malpas, 1998; Aricò et al., 1999, 2003).
Clinical aspects
The bones most commonly involved are skull, spine, lower extremity, pelvis, ribs and upper extremity (Table 8.1). Proximal long bones are commoner than distal (Figure 8.1) (Egeler et al., 1999), and hands and feet are rarely involved. In adults the flat bones are involved more frequently, with jaw involved in 30% (Baumgartner et al., 1997). The ribs accounted for only 6% of lesions in Baumgartner's series but was the second commonest bone affected in two other adult series (Kilpatrick et al., 1995; Islinger et al., 2000) (Figure 8.2). In the long bones diaphysis and metaphysis are equally involved, epiphysis is commonly spared, but epiphyseal location does not exclude LCH (Ghanem et al., 2003).
- Type
- Chapter
- Information
- Histiocytic Disorders of Children and AdultsBasic Science, Clinical Features and Therapy, pp. 154 - 173Publisher: Cambridge University PressPrint publication year: 2005
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