Revisiting acquired aplastic anaemia: current concepts in diagnosis and management.
Intern Med J
; 49(2): 152-159, 2019 02.
Article
em En
| MEDLINE
| ID: mdl-30324755
ABSTRACT
Acquired aplastic anaemia is a rare, serious, immunologically mediated bone marrow failure syndrome, characterised by marrow hypoplasia of varying severity and significant pancytopenia. Careful attention and investigation, including molecular testing, is required to confirm the diagnosis and exclude other mimicking conditions, such as inherited bone marrow failure syndromes. In a proportion of patients, the disease evolves to myelodysplasia or acute myeloid leukaemia and in some there is an association with paroxysmal nocturnal haemoglobinuria. The disease has a major impact on patient quality of life. Haemopoietic stem/progenitor cell transplantation for eligible patients with an available donor is the only current curative therapy. Other patients may receive immunosuppression, most commonly with anti-thymocyte globulin and cyclosporin. An initial response to immunosuppression is often encouraging, but relapse is common. Supportive care, including management of transfusion requirements and infections, is central to management. Promising new diagnostic tools and emerging therapies will likely transform approaches to this important, chronic and life-threatening condition.
Palavras-chave
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Anemia Aplástica
Tipo de estudo:
Diagnostic_studies
Limite:
Humans
Idioma:
En
Revista:
Intern Med J
Assunto da revista:
MEDICINA INTERNA
Ano de publicação:
2019
Tipo de documento:
Article
País de afiliação:
Austrália