Myeloid & Lymphoid disorders in practice - 2007

Comment: At last, the malady of mystery makes a name for itself
John Reilly
pp 2-2
Welcome to the second edition of Myeloproliferative Disorders in practice, a journal devoted to the myeloproliferative and myelodysplastic diseases. The Editorial Board has been encouraged by the positive feedback generated by the first issue and will try to maintain a balanced spectrum of topics, which will include a number suggested by our readers. The present edition contains articles on the management of polycythaemia, eosinophilia, iron chelation and primary myelofibrosis.
Simplifying PV diagnosis in the light of the JAK2 mutation
Mary Frances McMullin
pp 3-5
The first trials in polycythaemia vera (PV) were designed by the Polycythemia Vera Study Group (PVSG) in the USA in 1967. The scientists produced criteria that ensured all patients entering the trials had PV. As such, they were restrictive and excluded cases with early disease. In 2001 the World Health Organization (WHO) published a modified set of criteria that, in contrast, may not be strict enough, allowing a diagnosis of PV in patients with only mildly elevated haemoglobin (Hb) levels.
Stem cell transplantation in primary myelofibrosis
Damiano Rondelli and Ronald Hoffman
pp 6-8
Primary myelofibrosis (PMF) is a chronic myeloproliferative disorder (CMPD). Like the other CMPDs – such as chronic myeloid leukaemia (CML), essential thrombocythaemia (ET) and polycythaemia vera (PV) – PMF is a clonal haematological malignancy that originates at the level of the pluripotent haematopoietic stem cell (HSC). Activating mutations of the intracellular cytokine-signalling molecule JAK2 and/or the thrombopoietin receptor MPL have been reported in 90% of patients with PV and 50% of patients with ET and PMF. Among the Philadelphia chromosome-negative CMPDs, PMF is associated with the most serious prognosis.
The diagnosis and treatment of eosinophilic syndromes
Lisa M Baumann Kreuziger and David P Steensma
pp 9-12
Eosinophils are myeloid cells distinguished by abundant subcellular granules with avidity for acidic tissue dyes such as eosin. Eosinophilic granules contain cationic proteins (for example, major basic protein, eosinophil peroxidase and eosinophilic cationic protein), pro-inflammatory cytokines, haematopoietic growth factors and lipid mediators (such as leukotriene C4 and platelet-activating factor). Degranulation releases this potent brew and can destroy invading microbes, notably helminths, but excessive or inappropriate release contributes to the host tissue injury observed in a variety of immunological and haematological diseases.
Transfusional support and iron chelation in myelodysplasia
Sally Killick
pp 13-15
The myelodysplastic syndromes (MDSs) are clonal disorders of haematopoiesis. The syndromes share certain characteristic morphological abnormalities of the blood and bone marrow and also the risk of evolution to acute leukaemia, which varies depending on the subtype of MDS. The majority of patients are elderly and show symptoms of marrow failure despite increased marrow cellularity. The disease can be described according to the French–American–British (FAB) classification, or by the more recently introduced World Health Organization (WHO) classification.