Myeloid & Lymphoid disorders in practice - 2007

Comment: On myeloid disorders – and publishing the unthinkable
John Reilly
pp 2-2
The launch of a new journal is always an exciting event and so it is with Myeloproliferative Disorders in practice, a quarterly publication that will be sent free of charge to those with an interest in the field. It is anticipated that the readership will include not only consultant haematologists, but also specialist registrars, pharmacists and specialist haemato-oncology nurses. The aim is to summarise the current knowledge of both acute and chronic myeloproliferative diseases, including the myelodysplasias.

Identifying molecular defects in myeloproliferative disease
Paresh Vyas
pp 3-6
In the past decade, and especially the last three years, there have been impressive advances defining the molecular basis of the myeloproliferative disorders (MPDs). This article provides edited highlights in this field, beginning with chronic myeloid leukaemia (CML), which continues to provide a paradigm for how molecular insight influences clinical practice. The genetic mutations in the common non-Philadelphia-positive MPDs are then discussed (especially aspects of the JAK2 mutations in polycythaemia rubra vera [PV], essential thrombocythaemia [ET] and primary myelofibrosis [PMF]). Finally, molecular lesions in the less common MPDs are mentioned.

Update on novel drugs for use in acute myeloid leukaemia
Jonathan Kell
pp 7-9
For more than 30 years, the combination of daunorubicin and cytosine arabinoside (ara-C) has been the cornerstone of treatment for acute myeloid leukaemia (AML). Advances in dosing schedules and treatment intensity have resulted in complete remission (CR) rates in excess of 80%, although long-term survival is still only 45–50%. It is felt that combination chemotherapy has reached the maximum tolerated intensity and, spurred by the success of targeted agents such as imatinib in chronic myeloid leukaemia, similar small non-cytotoxic chemotherapeutics are under intense scrutiny in AML.

Essential thrombocythaemia: treatment for young patients
Susan E Robinson and Claire N Harrison
pp 10-13
The first description of essential thrombocythaemia (ET) was of ‘haemorrhagic thrombocythaemia’, where bleeding was associated with an abnormally high platelet count and megakaryocyte hyperplasia. Today, ET is seen as a relatively indolent myeloproliferative disorder (MPD) characterised by thrombotic or haemorrhagic complications and, in a proportion of patients over the long term, a risk of transformation to myelofibrosis (MF) or acute leukaemia. While the mean age at presentation is during the seventh decade, a significant proportion, particularly of female patients, are diagnosed at a much younger age.

Splenectomy in myelofibrosis: indications and risks
John Reilly
pp 14-15
Splenectomy can palliate the symptoms resulting from massive splenomegaly in patients with primary myelofibrosis (PMF). Even in the most experienced units, however, surgery is associated with significant morbidity and mortality. The need for meticulous pre- and postoperative care cannot be overstated. As a result, splenectomy should be restricted to carefully selected cases that present with refractory anaemia and/or thrombocytopenia, refractory haemolysis, severe symptomatic splenomegaly or significant portal hypertension.