CLINICAL PRACTICE GUIDELINES
**** MOH/P/PAK/ (GU)
DRAFT
MANAGEMENT OF TRANSFUSION DEPENDENT
THALASSAEMIA
Ministry of Health Malaysian Society of Academy of Medicine of
Malaysia Paediatric Haematology Malaysia
and Oncology
`
, Management of Transfusion Dependent Thalassaemia
This guideline is meant to be a guide for clinical practice, based on the best available evidence
at the time of development. Adherence to this guideline may not necessarily guarantee the best
outcome in every case. Every health care provider is responsible for the management of his/her
unique patient based on the clinical picture presented by the patient and the management
options available locally.
Review of the Guidelines
This guideline was issued in 2009 and will be reviewed in 2013 or sooner if new evidence
becomes available.
CPG Secretariat
Health Technology Assessment Section
Medical Development Division
Ministry of Health Malaysia
4th Floor, Block E1, Parcel E
62590 Putrajaya.
Electronic version available on the following website:
http://www.moh.gov.my
http://www.acadmed.org.my
ii
, Management of Transfusion Dependent Thalassaemia
GUIDELINE DEVELOPMENT AND OBJECTIVE
GUIDELINE DEVELOPMENT
The development group for this guideline consisted of paediatricians, paediatric haematologist
oncologists, paediatric endocrinologists, haematologists, pathologists, public health physicians,
a clinical geneticist, a family medicine specialist, a radiologist, a pharmacist and a nursing sister
from the Ministry of Health and Ministry of Higher Education, Malaysia.
Literature search was carried out at the following electronic databases: International Health
Technology Assessment Website, PUBMED, Cochrane Database of Systemic Reviews
(CDSR), Journal full text via OVID search engine, Database of Abstracts of Reviews of
Effectiveness, Cochrane Controlled Trials Registered, Science Direct and CINAHL. In addition,
the reference lists of studies selected for inclusion were scanned for relevant studies. Experts in
this field were also contacted to identify further studies.
The clinical questions were divided into major subgroups and members of the development
group were assigned individual topics within these subgroups. All literature retrieved were
appraised by at least two members using Critical Appraisal Skills Programme (CASP) checklists
and presented in the form of evidence tables and discussed during group meetings. All
statements and recommendations formulated were agreed by both the development group and
the review committee. Where the evidence was insufficient the recommendations were derived
by consensus of the development group and review committee.
The articles were graded using the US/Canadian Preventive Services Task Force Level of
Evidence (2001), while the grading of recommendation in this guideline was modified from the
Scottish Intercollegiate Guidelines Network (SIGN).
The draft guideline was posted on both the Ministry of Health Malaysia and Academy of
Medicine, Malaysia websites for comment and feedback. This guideline has also been
presented to the Technical Advisory Committee for Clinical Practice Guidelines, and the Health
Technology Assessment and Clinical Practice Guidelines Council, Ministry of Health Malaysia
for review and approval.
OBJECTIVES
GENERAL OBJECTIVES
To provide evidence-based guidance in the management of transfusion dependent
thalassaemia in paediatric and adult patients.
SPECIFIC OBJECTIVES
To provide guidance in diagnosing thalassaemia
To provide guidance in counseling and cascade screening
To provide guidance in blood transfusion for thalassaemia patients
To provide guidance in iron chelation therapy
To provide guidance in managing important complications in transfusion dependent
thalassaemia
iii
, Management of Transfusion Dependent Thalassaemia
It is not the objective of this guideline to cover
Management of thalassaemia patients who do not require regular blood transfusion
Prenatal & intrauterine management
Management of thalassaemia patients during pregnancy
Population screening
TARGET POPULATION
Adult and paediatric transfusion dependent thalassaemia patients
INCLUSION AND EXCLUSION CRITERIA
a) INCLUSION CRITERIA
Paediatric and adult transfusion dependent thalassaemia patients.
Definition
- Transfusion dependent thalassaemia patients are those who require life long regular
blood transfusions
b) EXCLUSION CRITERIA
Thalassaemia patients who do not require regular blood transfusion
CLINICAL QUESTIONS
Please refer to Appendix 2
TARGET GROUP/USER
All health care professionals/individuals who are involved in the management of transfusion
dependent thalassaemia patients including:-
Paediatrician
Physician
Haematologist
Pathologist
Radiologist
Family Medicine Specialist
Public Health Physician
Medical officer
General practitioner
Pharmacists
Dietician/Nutritionist
Nurses/Assistant Medical Officers
Laboratory personnel
Social workers
Patients and their families/caregiver
iv
**** MOH/P/PAK/ (GU)
DRAFT
MANAGEMENT OF TRANSFUSION DEPENDENT
THALASSAEMIA
Ministry of Health Malaysian Society of Academy of Medicine of
Malaysia Paediatric Haematology Malaysia
and Oncology
`
, Management of Transfusion Dependent Thalassaemia
This guideline is meant to be a guide for clinical practice, based on the best available evidence
at the time of development. Adherence to this guideline may not necessarily guarantee the best
outcome in every case. Every health care provider is responsible for the management of his/her
unique patient based on the clinical picture presented by the patient and the management
options available locally.
Review of the Guidelines
This guideline was issued in 2009 and will be reviewed in 2013 or sooner if new evidence
becomes available.
CPG Secretariat
Health Technology Assessment Section
Medical Development Division
Ministry of Health Malaysia
4th Floor, Block E1, Parcel E
62590 Putrajaya.
Electronic version available on the following website:
http://www.moh.gov.my
http://www.acadmed.org.my
ii
, Management of Transfusion Dependent Thalassaemia
GUIDELINE DEVELOPMENT AND OBJECTIVE
GUIDELINE DEVELOPMENT
The development group for this guideline consisted of paediatricians, paediatric haematologist
oncologists, paediatric endocrinologists, haematologists, pathologists, public health physicians,
a clinical geneticist, a family medicine specialist, a radiologist, a pharmacist and a nursing sister
from the Ministry of Health and Ministry of Higher Education, Malaysia.
Literature search was carried out at the following electronic databases: International Health
Technology Assessment Website, PUBMED, Cochrane Database of Systemic Reviews
(CDSR), Journal full text via OVID search engine, Database of Abstracts of Reviews of
Effectiveness, Cochrane Controlled Trials Registered, Science Direct and CINAHL. In addition,
the reference lists of studies selected for inclusion were scanned for relevant studies. Experts in
this field were also contacted to identify further studies.
The clinical questions were divided into major subgroups and members of the development
group were assigned individual topics within these subgroups. All literature retrieved were
appraised by at least two members using Critical Appraisal Skills Programme (CASP) checklists
and presented in the form of evidence tables and discussed during group meetings. All
statements and recommendations formulated were agreed by both the development group and
the review committee. Where the evidence was insufficient the recommendations were derived
by consensus of the development group and review committee.
The articles were graded using the US/Canadian Preventive Services Task Force Level of
Evidence (2001), while the grading of recommendation in this guideline was modified from the
Scottish Intercollegiate Guidelines Network (SIGN).
The draft guideline was posted on both the Ministry of Health Malaysia and Academy of
Medicine, Malaysia websites for comment and feedback. This guideline has also been
presented to the Technical Advisory Committee for Clinical Practice Guidelines, and the Health
Technology Assessment and Clinical Practice Guidelines Council, Ministry of Health Malaysia
for review and approval.
OBJECTIVES
GENERAL OBJECTIVES
To provide evidence-based guidance in the management of transfusion dependent
thalassaemia in paediatric and adult patients.
SPECIFIC OBJECTIVES
To provide guidance in diagnosing thalassaemia
To provide guidance in counseling and cascade screening
To provide guidance in blood transfusion for thalassaemia patients
To provide guidance in iron chelation therapy
To provide guidance in managing important complications in transfusion dependent
thalassaemia
iii
, Management of Transfusion Dependent Thalassaemia
It is not the objective of this guideline to cover
Management of thalassaemia patients who do not require regular blood transfusion
Prenatal & intrauterine management
Management of thalassaemia patients during pregnancy
Population screening
TARGET POPULATION
Adult and paediatric transfusion dependent thalassaemia patients
INCLUSION AND EXCLUSION CRITERIA
a) INCLUSION CRITERIA
Paediatric and adult transfusion dependent thalassaemia patients.
Definition
- Transfusion dependent thalassaemia patients are those who require life long regular
blood transfusions
b) EXCLUSION CRITERIA
Thalassaemia patients who do not require regular blood transfusion
CLINICAL QUESTIONS
Please refer to Appendix 2
TARGET GROUP/USER
All health care professionals/individuals who are involved in the management of transfusion
dependent thalassaemia patients including:-
Paediatrician
Physician
Haematologist
Pathologist
Radiologist
Family Medicine Specialist
Public Health Physician
Medical officer
General practitioner
Pharmacists
Dietician/Nutritionist
Nurses/Assistant Medical Officers
Laboratory personnel
Social workers
Patients and their families/caregiver
iv
