Haemophagocytic lymphohistiocytosis (HLH)
The study aimed to establish epidemiological data in the absence of other data from the United Kingdom, improve awareness and diagnosis and advise on the management.
Lead investigator
Professor S Strobel
About the study
At the time of the study the term haemophagocytic lymphohistiocytosis (HLH) included the disease previously known as familial erythrophagocytic lymphohistiocytosis and cases associated with infection, including virus-associated haemophagocytic syndrome. The distinction was misleading; cases that are likely to have a genetic basis may not have a positive family history. Familial HLII can be triggered by infection, including infection with viruses.
The study aimed to:
- Establish epidemiological data in the absence of other data from the United Kingdom (only one retrospective survey has been reported, from Sweden).
- Improve awareness and diagnosis. Although there is no diagnostic test, the syndrome is fairly easy to recognise if clinicians are aware of it.
- Advise on management. The research team felt that the advent of bone marrow transplantation had made this disease potentially curable. The study coordinators had been able to advise on diagnosis and current management based on an increasing number of patient histories and contact with the reporting practitioners.
- Research on aetiology and pathogenesis.
This study helped to establish a network of clinical and basic research.
Duration
September 1991 – August 1994
Published papers
Support group
The Histiocytosis Research Trust
Web: http://www.hlhsupport.org Email: info@hlhsupport.org