Comment: William Dameshek and the myeloproliferative disorders - 60 years on
John Reilly
pp 2-2
One of the most widely quoted publications in the field of the myeloproliferative neoplasms is a rather brief editorial by William Dameshek, published in 1951 in the journal Blood. But who was Dameshek and why should his musings, entitled somewhat prosaically, ‘Some speculations on the myeloproliferative syndromes’, have commanded such lasting attention?
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Arsenic: poison or cure?
Jonathan Kell
pp 3-5
Historically, treatments for the myelodysplastic syndromes (MDS) have been limited to supportive measures with blood product and antibiotic support. A new era has dawned, in which we see novel approaches to this syndrome of diseases, with low-dose chemotherapy, hypomethylating agents and other disease-modifying agents showing promising results in subsets of patients. One of these agents is arsenic, well known for its role in many Agatha Christie and real-life murder plots, green wallpaper and, allegedly, in Napoleon’s demise, as well as in the treatment of acute promyelocytic leukaemia (APL). Promising results have also been published for MDS and myeloma.
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Diagnosis and prognosis of myelodysplastic syndromes: part one
Lynn Quek and Paresh Vyas
pp 6-8
The myelodysplastic syndromes (MDS) are a group of haematological malignancies characterised by ineffective haematopoiesis in at least one of the lineages of bone marrow. They are clonal in nature, and are likely to originate from an MDS stem cell. MDS occurs de novo, secondary to radiation or chemical toxicity, or following congenital bone marrow failure. However, the majority of cases remain idiopathic in aetiology.
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Molecular monitoring in chronic myeloid leukaemia
Sarah McCarron and Stephen Langabeer
pp 9-11
Chronic myeloid leukaemia (CML) is a clonal disease of haematopoietic stem cells characterised by a reciprocal translocation between chromosomes 9 and 22 that results in the Philadelphia (Ph) chromosome. This translocation fuses sequences of the BCR gene on chromosome 22 to the ABL1 gene on chromosome 9 to create the BCR-ABL1 oncogene with the resultant protein displaying enhanced tyrosine kinase activity that propagates the CML phenotype via numerous deregulated signalling pathways involved in proliferation, apoptosis, cellular adhesion and genomic stability.
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Hypoplastic myelodysplastic syndrome: classification and treatment
Austin G Kulasekararaj, Victoria J Tindell, Modupe O Elebute and Ghulam J Mufti
pp 12-14
The myelodysplastic syndromes (MDS) are a heterogeneous group of clonal stem cell disorders characterised by ineffective haematopoiesis, dysplastic marrow, cytopenia(s) due to impaired blood cell production and a variable propensity for leukaemic transformation. Although the bone marrow of an MDS patient is usually hypercellular or normocellular, a subset of patients at presentation has reduced bone marrow cellularity, even if age is taken into consideration. This subset of MDS patients may be difficult to distinguish from aplastic anaemia (AA).
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Hydroxycarbamide-related fever: a rare side-effect
Richard Went
pp 15-15
The potential of hydroxycarbamide (HC) to cause significant fever in myeloproliferative neoplasms (MPNS) is well established. It is considered severe enough to be one of the indications to change to a second-line agent in the management of essential thrombocythaemia (ET). However, our understanding of this complication remains poor.
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