Epidemiology of haemoglobin disorders in Europe: an overview

Abstract

There is a strong case for pan-European collaboration on haemoglobin disorders to share policies, standards and the instruments required to support them. These include methods for needs assessment, service standards, education and information strategies and materials, and methods for evaluating service delivery.

Sickle cell disease as a paradigm of immigration hematology: new challenges for hematologists in Europe

“…The highest frequency of sickle cell disease remains in tropical regions, particularly sub-Saharan Africa, India and the Middle East. 4 Migration of substantial populations from these high prevalence areas to low prevalence countries in Europe began more than 50 years ago but has dramatically increased with greater geographic mobility over the last 10-15 years [5][6][7] such that in some European countries sickle cell disease has now overtaken more familiar genetic disorders such as hemophilia and cystic fibrosis. 7,8 In most endemic countries there are no accurate figures of disease prevalence.…”

“…4 Migration of substantial populations from these high prevalence areas to low prevalence countries in Europe began more than 50 years ago but has dramatically increased with greater geographic mobility over the last 10-15 years [5][6][7] such that in some European countries sickle cell disease has now overtaken more familiar genetic disorders such as hemophilia and cystic fibrosis. 7,8 In most endemic countries there are no accurate figures of disease prevalence. A recent WHO report estimated that around 20 per 1,000 births in Nigeria were affected by sickle cell anemia, giving a total of 150,000 affected children born every year in Nigeria alone.…”

“…9 There is marked variation in carrier frequency, not only between countries (Table 1) but also between regions within individual countries, the Baamba tribe in western Uganda, for example, having a carrier frequency of 45%. 7,9,10 This variation means that accurate data are essential for characterizing the burden of disease and hence resource allocation, particularly in poorly-resourced countries. Some progress has already been made in thalassaemia through micromapping 1 and in sickle cell disease through the pioneering work of Graham Serjeant in Jamaica, 11 but there remains a huge task to complete.…”

“…There have been many national and local initiatives in non-endemic countries to obtain accurate, up-todate measures of the changing prevalence of sickle cell disease, 5,7,12,13,15,[17][18][19][20] although attempts to establish comprehensive national registries have so far largely failed, despite individual efforts, because of insufficient funding. 19,21 An alternative approach, which provides an essential starting point for planning purposes, is to estimate the carrier frequency using national population data including information about country of origin.…”

Thalassaemia Prevention: Bangladesh Perspective - A Current Update

“…In the late 1970s pilot population programs directed to prevent β-thalassaemia major by carrier screening, counseling and prenatal diagnosis started in several at-risk populations in the Mediterranean area (Cyprus, Sardinia, several regions of Continental Italy). 12 At present, several countries like Italy, Greece, Cyprus, UK, France, Iran, Thailand, Australia, Singapore, Taiwan, Hong Kong, Cuba and also in Northern Europe ( Netherlands, Belgium and Germany) 13 have set up comprehensive national thalassaemia prevention program. Cyprus and Sardinia both are the unique examples for successful thalassaemia prevention.…”