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
16 - The histopathology of hemophagocytic lymphohistiocytosis
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
Although the earliest report of the inherited condition, now known as familial hemophagocytic lymphohistiocytosis (FHL) (OMIM 603552, 603553, 602782, 170280), is traditionally attributed to Farquhar and Claireaux (1952), it was not until 1995 that a logical framework for the understanding of these conditions was provided (Henter and Elinder, 1995; Henter, 2002). According to this schema, hemophagocytic lymphohistiocytosis (HLH) with an underlying genetic defect is regarded as ‘primary HLH’. All other forms are reactive, examples of the macrophage activation syndrome, and hence, ‘secondary HLH’. The ‘contemporary classification of histiocytic disorders’ (Favara et al., 1997) lists primary HLH as including a clearly familial form as well as a sporadic form without a clear family history, nonetheless thought to be genetic, and secondary hemophagocytic syndromes that are infection or malignancy associated. Curiously, it is not the histiocyte (monocyte-macrophage) that is abnormal in either form. In primary HLH there is a defect in the control of the inflammatory process, such as perforin mutations, that leads to the activation and accumulation of macrophages. In secondary HLH, the macrophage activation appears to be driven by cytokines following a trigger. It is however, becoming apparent that even in secondary, reactive macrophage activation type HLH, there may be an inherited predisposition in the form of reactive versus non-reactive polymorphisms for certain receptors and response molecules such as IL-10, IL-12 and IL-18 (Lammas et al., 2002; Fujihara et al., 2003; Goldstein et al., 2003).
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- Histiocytic Disorders of Children and AdultsBasic Science, Clinical Features and Therapy, pp. 321 - 336Publisher: Cambridge University PressPrint publication year: 2005
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