Myeloid & Lymphoid disorders in practice - 2009

Comment: Molecular analyses in MPNS – the need for standardisation
John Reilly
pp 2-2
During the last decade, molecular testing has become routine for the diagnosis and management of the myeloproliferative neoplasms (MPNs). The diagnosis of the Philadelphia-negative MPNs, for example, has been revolutionised by the discovery of the JAK2 V617F mutation, as well as the functionally similar JAK2 exon 12 and MPL W515 mutations, all of which are regarded as major diagnostic criteria by the WHO.
Making the case for top-up payments in haematology
Mike Galloway
pp 3-5
The NHS in the UK continues to differentiate from the pluripotential stem cell that was the NHS before 1998 when the current process of devolution commenced. The result is that the four countries of the UK have evolved very different approaches to the management of health services. Scotland and Wales have adopted a cooperative single system of delivery.
Treating acute myeloid leukaemia in pregnancy
Saran Nicholas and Jonathan Kell
pp 6-8
Acute myeloid leukaemia (AML) is an aggressive malignant disorder of the blood and bone marrow, characterised by the accumulation of immature and dysfunctional white cells with concomitant suppression and disruption of normal haematopoiesis. It demands urgent and vigorous treatment with intensive chemotherapy to effect a cure. Occasionally, AML is diagnosed during pregnancy, with an estimated incidence of one in 10,000 pregnancies, presenting both the patient and the physician with difficult choices with regard to treatment.
Benefits and indications for combination therapy in MPNs
Natasha Wiles and Claire N Harrison
pp 9-11
Hydroxycarbamide (also known as hydroxyurea) is the treatment of choice for most patients with essential thrombocythaemia (ET) and polycythaemia vera (PV) who are at high risk of thrombosis, although the definition of high-risk disease is variable in the literature. There is probably more concordance with regard to the definition of high-risk ET.
A review of the clinical use of deferasirox
Josh Wright and Nina Farquharson
pp 12-14
Iron overload as a consequence of multiple red cell transfusions is a well-recognised complication of thalassaemia major and other congenital anaemias, as well as myelodysplastic syndrome (MDS). Once the usual iron transport mechanisms become saturated, increasing levels of non-transferrin-bound iron (NTBI) lead to the formation of toxic hydroxyl free radicals. Tissue iron accrual eventually leads to organ dysfunction, typically in the heart, liver and endocrine organs. Iron-induced heart failure and arrhythmia remain the most common cause of mortality in beta (ß) thalassaemia major, accounting for up to 70% of premature deaths.