Guidelines for the diagnosis and management of adult aplastic anaemia
Guidelines for the diagnosis and management of adult aplastic anaemia - British Society for Haematology (BSH)
This is a comprehensive but pragmatic new guideline and focuses on the accurate diagnosis, delineation of severity types and subsequent management of this rare haematological condition.
The guideline emphasises the importance at the diagnostic stage of considering and testing for inherited bone marrow failure syndromes such as Fanconis anaemia and dyskeratosis congenita, as it is increasingly recognised that patients may present in adulthood with few or no somatic features. There is a comprehensive table with recommended baseline investigations, bone marrow findings and alternative diagnoses to consider which may be confused with aplastic anaemia.
Treatment is divided into supportive and specific, the latter providing up to date recommendations for immunosuppressive treatment and who should be considered for allogeneic stem cell transplantation. A treatment algorithm is provided and advice regarding timing of donor searches.
Pragmatic definitions are provided for treatment responses and outcomes and finally there are separate concise sections on management in pregnancy , of the more frail and elderly patient and the relationship between paroxysmal nocturnal haemoglobinuria and aplastic anaemia at diagnosis and more generally.