A MULTICENTRE, RETROSPECTIVE STUDY OF EPIDEMIOLOGY AND OUTCOME OF APLASTIC ANAEMIA AMONG ADULT POPULATION IN SABAH AND SARAWAK FROM YEAR 2006 TO 2017
Introduction: Aplastic anaemia (AA) is a rare disorder of bone marrow failure, characterized by bone marrow hypocellularity with pancytopenia. The annual incidence rates of AA in Asia are observed to be two to three times higher than Europe and North America. Since the introduction of immunosup...
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| Main Authors: | , , , , , , , , |
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| Format: | Article |
| Language: | English |
| Published: |
Malaysian Medical Association
2024
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| Subjects: | |
| Online Access: | http://ir.unimas.my/id/eprint/46927/1/A%20multicentre.pdf http://ir.unimas.my/id/eprint/46927/ https://www.e-mjm.org/2024/v79n6/aplastic-anaemia.pdf |
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| Summary: | Introduction: Aplastic anaemia (AA) is a rare disorder of
bone marrow failure, characterized by bone marrow
hypocellularity with pancytopenia. The annual incidence
rates of AA in Asia are observed to be two to three times
higher than Europe and North America. Since the
introduction of immunosuppressive therapy (IST) and of
allogenic stem cell transplant (SCT), the outcome of severe
AA has significantly improved. We conducted a 12-year
multi-centre retrospective study among the adult AA
population in Sabah and Sarawak.
Materials and methods: A total of 119 AA patients had been
identified from hospital records of the involved sites, namely
Queen Elizabeth Hospital in Sabah, Sarawak General
Hospital, Sibu Hospital, Miri Hospital and Bintulu Hospital in
Sarawak from Jan 2006 to Dec 2017.
Results: The median age at diagnosis was 46 years, and
native ethnic group from Sabah, Kadazan-Dusun, recorded
the highest percentage of 41.2%, which could be explained
by higher frequency of HLA-DRB1*15:01, an alelle linked to
increased risk of AA, among this ethnic group. The majority
of patients (59.7%) received cyclosporine (CsA) as
monotherapy or in combination with other non-IST agents
such as danazol, which was instituted in 52.1% of the
patients, while a third of them (33.7%) received antithymocyte globulin (ATG) therapy with or without CsA, and
12.4% underwent allogenic SCT. The five-year overall
survival (OS) for all AA patients was 76.1%. Elderly patients
>60 years old and those with severe disease had more
inferior 5-year survival.
Conclusion: A prospective study is warranted to determine
the true incidence rate, epidemiological distributions,
treatment outcome and overall survival of AA patients in
Malaysia. Establishment of allogenic SCT in East Malaysia is
imperative to make this curative therapy more accessible to
patients with severe disease and improve the outcome. |
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