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Observational study on the current status of thalassaemia in Malaysia: a report from the Malaysian Thalassaemia Registry

Mohd Ibrahim H 1 , Muda Z 2 , Othman IS 2 , Mohamed Unni MN 2 , Teh KH 2 , Thevarajah A 3 Show all authors , Gunasagaran K 3 , Ong GB 4 , Yeoh SL 5 , Muhammad Rivai A 6 , Che Mohd Razali CH 7 , Din ND 8 , Abdul Latiff Z 9 , Jamal R 10 , Mohamad N 11 , Mohd Ariffin H 12 , Alias H 13

Affiliations 

  • 1 Division for Research and Technical Support, Ministry of Health Malaysia, Putrajaya, Wilayah Persekutuan Putrajaya, Malaysia
  • 2 Department of Paediatrics, Hospital Tunku Azizah, Kuala Lumpur, Wilayah Persekutuan Kuala Lumpur, Malaysia
  • 3 Department of Paediatrics, Sabah Women & Children Hospital, Likas, Kota Kinabalu, Sabah, Malaysia
  • 4 Department of Paediatrics, Hospital Umum Sarawak, Kuching, Sarawak, Malaysia
  • 5 Department of Paediatrics, Hospital Pulau Pinang, Georgetown, Pulau Pinang, Malaysia
  • 6 Department of Paediatrics, Hospital Raja Permaisuri Bainun, Ipoh, Perak, Malaysia
  • 7 Department of Paediatrics, Hospital Sultan Ismail, Johor Bharu, Johor, Malaysia
  • 8 Department of Paediatrics, Hospital Sultanah Nur Zahirah Kuala Terengganu, Kuala Terengganu, Terengganu, Malaysia
  • 9 Department of Paediatrics, UKM Medical Centre, The National University of Malaysia, Jalan Yaacob Latif, Cheras, Kuala Lumpur, Malaysia
  • 10 UKM Medical Molecular Biology Institute, Jalan Yaacob Latif, Cheras, Kuala Lumpur, Malaysia
  • 11 Department of Paediatrics, Hospital Universiti Sains Malaysia, Kota Bahru, Kelantan, Malaysia
  • 12 Department of Paediatrics, University of Malaya Medical Centre, Kuala Lumpur, Wilayah Persekutuan Kuala Lumpur, Malaysia
  • 13 Department of Paediatrics, UKM Medical Centre, The National University of Malaysia, Jalan Yaacob Latif, Cheras, Kuala Lumpur, Malaysia midalias@ppukm.ukm.edu.my
BMJ Open, 2020 06 29;10(6):e037974.
PMID: 32601117 DOI: 10.1136/bmjopen-2020-037974

Abstract

OBJECTIVE: Thalassaemia is the most common inherited blood disorder in Malaysia. This study aims to report the current status of thalassaemia in Malaysia and provide a comprehensive understanding of the disease through data obtained from the Malaysian Thalassaemia Registry.

DESIGN: Data were extracted from the Malaysian Thalassaemia Registry, a web-based system accessible to enrolled users through www.mytalasemia.net.my.

SETTING: The Malaysian Thalassaemia Registry data was recorded from reports obtained from 110 participating government and university hospitals in Malaysia.

PARTICIPANTS: The patients were those attending the 110 participating hospitals for thalassaemia treatment.

INTERVENTION: Data were collected from the Malaysian Thalassaemia Registry from 2007 until the fourth quarter of 2018.

PRIMARY OUTCOME MEASURE: 7984 out of 8681 patients with thalassaemia registered in the Malaysian Thalassaemia Registry were reported alive.

RESULTS: Majority of the patients were reported in the state of Sabah (22.72%); the largest age group affected was 5.0-24.9 years old (64.45%); the largest ethnic group involved was Malay (63.95%); and the major diagnosis was haemoglobin E/β-thalassaemia (34.37%). From the 7984 patients, 56.73% were on regular blood transfusions and 61.72% were on chelation therapy. A small fraction (14.23%) has undergone splenectomy, while the percentage of patients with severe iron overload (serum ferritin ≥5000 µg/L) reduced over time. However, cardiac complications are still the main cause of death in patients with thalassaemia.

CONCLUSION: Data gathered into the registry can be used to understand the progression of the disorder, to monitor iron overload management and to improve the outcomes of treatment, to enhance preventive strategies, reduce healthcare burden and improve the quality of life. Sustainability of the Malaysian Thalassaemia Registry is important for surveillance of thalassaemia management in the country and help the national health authorities to develop more effective policies.

* Title and MeSH Headings from MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.

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