Myeloid & Lymphoid disorders in practice - 2009


Comment: JAK2 and myeloproliferative neoplasms - an evolving story
John Reilly
pp 2-2
The identification of JAK2 mutations has greatly increased our understanding of the myeloproliferative neoplasms (MPNs), and has helped to catapult these diseases to the forefront of haematological research. However, many unanswered questions still remain. What induces the JAK2 mutation in the first place? Why do some families have a high incidence of MPNs? What additional mutations might co-operate, since JAK2 mutations alone cannot account for all the known pathophysiological features of these disorders?
Allogeneic SCT in chronic phase CML: who should you transplant?
Jiri Pavlu and Jane F Apperley
pp 3-5
The past decade has witnessed a dramatic change in the management of chronic myeloid leukaemia (CML), such that the majority of patients initially receive therapy with tyrosine kinase inhibitors (TKIs) rather than being offered early stem cell transplantation (SCT). However, a significant proportion of these patients will fail to respond and/or will develop intolerance to TKIs, so SCT remains an effective therapeutic solution. The timing of SCT in CML remains a complex management decision.
How to manage palpitations when treating ET
Manuel Martínez-Sellés
pp 6-7
Palpitations can be caused by both cardiac and non-cardiac conditions. In this article I will review how to identify the causes of palpitations and manage these patients. Palpitations associated with certain cytoreductive therapies are usually benign and subside over time. In most cases, management may require only a review of lifestyle, patient education and reassurance.
The diagnosis and management of APL
David Grimwade
pp 8-14
Acute promyelocytic leukaemia (APL) is one of the most common subtypes of acute myeloid leukaemia (AML), accounting for 10–15% of cases. APL has been of particular interest as the first form of leukaemia for which molecularly targeted therapies (all-trans-retinoic acid [ATRA] and arsenic trioxide [ATO]) have been successfully used in clinical practice, transforming its management and making it one of the most prognostically favourable subtypes of AML, with >=70% of patients in remission at five years.
Is chronic myeloid leukaemia curable with drugs?
Mhairi Copland
pp 12-14
Chronic myeloid leukaemia (CML) is a clonal haemopoietic stem cell (HSC) disorder which results from a reciprocal translocation between chromosomes 9 and 22 t(9:22)(q34;q11). This produces a shortened chromosome 22, called the Philadelphia chromosome, and a fusion gene product, BCR-ABL, which is a constitutively activated tyrosine kinase. BCR-ABL has transforming activity both in vitro and in vivo, and is associated with inhibition of apoptosis, increased cell proliferation and alteration of cellular adhesion.
Response to peginterferon alfa – one patient's experience
Catherine J Rea
pp 15-15
A Phase II trial examining the effects of de novo peginterferon alfa-2a as treatment for patients with polycythaemia vera has recently been completed. Forty patients were included in the study. Peginterferon alfa-2a was well tolerated and produced significant haematological and molecular responses. This case study examines the experience of an individual with a myeloproliferative neoplasm receiving treatment with peginterferon alfa-2a.

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