Myeloid & Lymphoid disorders in practice - 2008

Comment: Clinical trials in CMPDs – the stage is set
John Reilly
pp 2-2
What better evidence for the phenomenal rate of progress in the field of chronic myeloproliferative disorders (CMPDs) than the contents list for this issue?

Using JAK2 inhibitors in myeloproliferative neoplasms
Laurent Knoops, Carmen C Diaconu, and Stefan N Constantinescu
pp 3-6
In 2005, several groups demonstrated that an acquired mutation in JAK2 that leads to the substitution of a phenylalanine for a valine at the amino acid position 617 (JAK2 V617F) is present in 95% of cases of polycythaemia vera (PV) and about 50% of cases of essential thrombocythaemia (ET) and primary myelofibrosis (PMF).

Nilotinib: a second-generation BCR-ABL kinase inhibitor
Mhairi Copland
pp 7-8
In the last five years, the BCR-ABL tyrosine kinase inhibitor imatinib mesylate (IM) has become firstline treatment for patients with chronic myeloid leukaemia (CML). IM has shown efficacy in all phases of CML, but is most effective in chronic phase, with a complete cytogenetic response (CCR) rate of 87% and progression-free survival of 93% at 60 months’ follow-up.

The diagnosis and treatment of systemic mastocytosis
Alfonso Quintás- Cardama and Srdan Verstovsek
pp 9-11
The term systemic mastocytosis (SM) denotes a group of heterogeneous haematopoietic neoplasms characterised by excessive proliferation of neoplastic mast cells (MCs) that accumulate in one or more organ system. SM as a single entity is an extremely rare disorder whose incidence is unknown.

The role of the nurse-led clinic in the long-term care of patients with MPDs
Michelle Taylor and E Sarah Green
pp 12-14
The myeloproliferative disorders (MPDs) – polycythaemia vera, essential thrombocythaemia (ET) and primary myelofibrosis – are a group of chronic haematological disorders that require long-term follow-up. Patients need counselling and support at the time of diagnosis, and regular review of their symptoms, signs and full blood count (FBC), with venesection and/or introduction/adjustment of therapy, including myelosuppressive medication. Follow-up is crucial to ensure recognition of side-effects or complications of therapy, and disease evolution.

ET – a difficult case
John Reilly
pp 15-15
The results of the Medical Research Council Primary Thrombocythaemia 1 (MRC PT1) study suggest that patients with risk factors for essential thrombocythaemia (ET) – age >60 years, platelet count =1,500 x 10⁹/l, or a history of diabetes, thrombosis or hypertension – are best treated with hydroxycarbamide (HC) and lowdose aspirin as firstline therapy. However, as highlighted in this case history, some patients may need combination therapy to achieve a normal platelet count and rare cases may still present with ongoing vascular complications, despite adequate treatment.