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Title: | Revisiting acquired aplastic anaemia: current concepts in diagnosis and management. | Authors: | Firkin F.;Szer J.;Blombery P.;McQuilten Z.K. ;Hiwase D.;Cole-Sinclair M.F.;Clucas D.B.;Fox L.C.;Wood E.M.;Hong F.S.;Gibson J.;Bajel A. | Institution: | (Clucas) Department of Clinical Haematology, Alfred Hospital, Monash University, Melbourne, VIC, Australia (Fox) Department of Clinical Haematology, Epworth Hospital, Monash University, Melbourne, VIC, Australia (Fox, Wood, McQuilten) Transfusion Research Unit, Department of Epidemiology and Preventive Medicine, Monash University, Melbourne, VIC, Australia (Wood, McQuilten) Department of Clinical Haematology, Monash Medical Centre, Melbourne, VIC, Australia (Hong) Department of Clinical Haematology, The Northern Hospital, Melbourne, VIC, Australia (Hong) Clinical Services and Research, Australian Red Cross Blood Service, Melbourne, VIC, Australia (Gibson) Institute of Haematology, Royal Prince Alfred Hospital, Adelaide, SA, Australia (Gibson) The University of Sydney, Sydney, SA, Australia (Bajel, Szer) Integrated Haematology Service, Victorian Comprehensive Cancer Centre (The Royal Melbourne Hospital/Peter MacCallum Cancer Centre), Melbourne, VIC, Australia (Blombery) Molecular Haematology Laboratory, Peter MacCallum Cancer Centre, Melbourne, VIC, Australia (McQuilten) Australian and New Zealand Intensive Care Research Centre, Monash University, Melbourne, VIC, Australia (Hiwase) Department of Clinical Haematology, Royal Adelaide Hospital, Adelaide, SA, Australia (Hiwase) The University of Adelaide, Adelaide, SA, Australia (Firkin, Cole-Sinclair) Haematology Department, St Vincent's Hospital, Melbourne, VIC, Australia (Firkin, Cole-Sinclair) The University of Melbourne, Melbourne, VIC, Australia | Issue Date: | 19-Feb-2019 | Copyright year: | 2019 | Publisher: | Blackwell Publishing | Place of publication: | Australia | Publication information: | Internal Medicine Journal. 49 (2) (pp 152-159), 2019. Date of Publication: Februaryy 2019. | Journal: | Internal Medicine Journal | 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.Copyright © 2018 Royal Australasian College of Physicians | DOI: | http://monash.idm.oclc.org/login?url=http://dx.doi.org/10.1111/imj.14140 | PubMed URL: | 30324755 [http://www.ncbi.nlm.nih.gov/pubmed/?term=30324755] | ISSN: | 1444-0903 | URI: | https://repository.monashhealth.org/monashhealthjspui/handle/1/36226 | Type: | Review | Type of Clinical Study or Trial: | Review article (e.g. literature review, narrative review) |
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