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
2 - The diagnostic histopathology 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
Langerhans cell (LC) disease covers a wide range of clinical presentations with peaks of incidence in early and later life. What ties these disparate conditions together is their histopathology, which has as its bedrock the identification, in the tissues or fluids, of a population of abnormal Langerhans cell histiocytosis cells (LCH cells). Like all other laboratory tests, biopsy pathology must be subjected to the rules of sensitivity and specificity. Sensitivity relates to the incidence of false negative samples. Sampling is always an issue in biopsy pathology, but considerations of sensitivity relate to those features that are essential to the diagnosis so that ideally, all patients with LCH will be identified. Specificity in a diagnostic biopsy is concerned with false positive results, and deals with the differential diagnosis of those conditions most likely to be mistaken for LCH. Since LCH involves a multitude of different anatomical sites, each of which has unique issues of access, intrinsic anatomy and cell populations, as well as the other lesions characteristic to that site, the sensitivity and specificity of LCH diagnosis must be considered for each site.
The diagnostic criteria for LCH have been a moving target. Birbeck and colleagues first described intracytoplasmic inclusions in dermal LCs (Birbeck et al., 1961). Soon after Birbeck granules were found in histiocytosis X (Basset and Nezelof, 1966), tying histiocytosis X and dermal LCs together (Nezelof et al., 1973). Later CD1a was identified as a useful diagnostic marker for LCs and later for histiocytosis X (Murphy et al., 1983).
- Type
- Chapter
- Information
- Histiocytic Disorders of Children and AdultsBasic Science, Clinical Features and Therapy, pp. 14 - 39Publisher: Cambridge University PressPrint publication year: 2005
- 24
- Cited by