Comment: Chronic myeloproliferative disorders take centre stagev
John Reilly
pp 2-2
The Congress of the European
Hematology Association continues to increase in popularity and stature, with the 2008 meeting in Copenhagen attracting nearly
7,000 delegates. Despite the city’s tradition of hospitality and its many cultural distractions, the educational programmes were well
attended, especially the session on
myeloproliferative disorders (MPDs).
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The current status of biphenotypic leukaemia
Ronan Swords, Yesid Alvarado and Francis J Giles
pp 3-5
In the majority of cases, acute leukaemia can easily be classified. However, definitive classification becomes difficult in cases where
blasts express antigens of more than one lineage. Biphenotypic leukaemia (BAL) is a subset of acute
leukaemia characterised by the presence of two or more blast populations of different lineages.
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Chronic myeloproliferative disorders in childhood
Georgina W Hall
pp 6-7
Although myeloproliferative disorders (MPDs) occur in children, they are rare and, when they
are finally recognised, management can be difficult. The true incidence and natural history of the three main chronic MPDs (CMPDs) –
polycythaemia vera (PV), essential thrombocythaemia
(ET) and primary myelofibrosis (PMF) – in children is unknown. However, ET is thought to be the most common of the three in
childhood, with an estimated frequency of about one per million.
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How will we afford new cancer drugs?
Steve Williamson
pp 8-11
There are many new and exciting drugs available or coming soon for myeloproliferative disorders
(MPDs). This is great news for both patients and prescribers but, unfortunately, most of these new
agents are expensive and gaining access to them is not straightforward. Understanding of the effect of health economics is becoming essential when seeking to use these medicines.
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Lenalidomide for MDS with deletion of 5q
Jonathan Kell
pp 12-14
Myelodysplastic syndrome (MDS) is
a heterogeneous collection of disorders characterised by ineffective haematopoiesis,
hypercellular bone marrow, characteristic dysplastic changes in cellular morphology and
peripheral cytopenia. These disorders differ in their propensity to progress to acute myeloid
leukaemia (AML) and in their prognosis. MDS with an isolated partial deletion of the long arm
of chromosome 5 was recognised as a separate entity by van den Berghe and is characterised by
a severe, refractory, macrocytic anaemia, thrombocytosis,
typically hypolobulated megakaryocytes,
a female preponderance and a generally favourable outlook.
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Budd–Chiari syndrome – a delayed diagnosis
Nauman M Butt
pp 15-15
Haematological disorders, in particular the myeloproliferative disorders (MPDs), are increasingly recognised as being the most
common aetiological factor associated with the development of Budd–Chiari syndrome (BCS).
However, on presentation, the typical radiological features of BCS and the peripheral blood features
of MPD may be absent or they may be misinterpreted, which can delay the diagnosis.
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