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
19 - Secondary haemophagocytic syndromes associated with rheumatic diseases
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
Macrophage activation syndrome (MAS) is a term used by rheumatologists to describe a form of secondary haemophagocytic lymphohistiocytosis (HLH) in association with chronic rheumatic diseases of childhood. It occurs most commonly with systemic-onset juvenile rheumatoid arthritis (SoJRA), but has also been reported with other rheumatic diseases. MAS manifestations may be seen at the onset of the rheumatic disease, during periods of active disease or even when the underlying rheumatic disease is quiescent.
Historical perspective and issues with nomenclature
The earliest description of MAS in the paediatric rheumatology literature may be the report of deaths from hepatic failure in patients with SoJRA at the first American Rheumatism Association(ARA) conference on rheumatic diseases of childhood (Boone, 1977). In 1983, Silverman described seven patients with ‘consumptive coagulopathy’ in children with SoJRA, either following the second dose of intramuscular gold or in the face of active disease (Silverman et al., 1983). The prominent clinical feature seen was a coagulopathy resembling disseminated intravascular coagulation (DIC), which most likely was a manifestation of secondary HLH. Subsequently, Jacobs reported five children (four with SoJRA, one with polyarticular juvenile rheumatic arthritis (JRA)) with a fatal outcome from a similar coagulopathy following the second gold injection (Jacobs et al., 1984). Hadchouel in 1985 described seven patients with SoJRA who developed a clinical syndrome characterized by haemorrhagic and neurological manifestations and hepatic, haematological and metabolic derangements (Hadchouel et al., 1985).
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- Chapter
- Information
- Histiocytic Disorders of Children and AdultsBasic Science, Clinical Features and Therapy, pp. 380 - 395Publisher: Cambridge University PressPrint publication year: 2005
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