Myeloid & Lymphoid disorders in practice - 2012


Comment: What, another prognostic score?
John Reilly
pp 2-2
Haematologists will be familiar with the plethora of prognostic scoring systems that are available to support the management of patients with primary myelofibrosis (PMF), myelodysplastic syndrome (MDS) and chronic myeloid leukaemia (CML). However, the utility of each schema is often short-lived, as more robust modifications and refinements are published, while a few gather dust as a result of therapeutic advances. The previously useful Hasford and Sokal scores for CML, for example, now appear inadequate for predicting overall survival in the tyrosine kinase inhibitor era.
Myelofibrosis symptoms and management
Holly L Geyer, Robyn M Emanuel and Ruben A Mesa
pp 3-5
Myelofibrosis (MF) is recognised as a Philadelphia chromosome-negative myeloproliferative neoplasm (MPN) that encompasses the phenotypically indistinct primary MF (PMF), postpolycythemia vera (PV) MF and post-essential thrombocythemia (ET) MF. Historically, this malignancy has challenged physicians with its high degree of symptomatology and dismal prognosis. In this article, we review the manifestations, complications and available treatment options for the symptoms expressed by MF patients.
The chequered history of gemtuzumab ozogamicin
Jonathan Kell
pp 6-8
One hundred years ago, Paul Ehrlich was breaking new ground in the treatment of infections and staining histological sections with various compounds with selective properties. His research gave birth to the idea of the magic bullet, a concept that has remained something of a holy grail in medicine for a century. The idea of involving the immune system in the control of cancer is also rapidly approaching late middle age, while antibody therapies are routine in the clinic.
Myeloid sarcoma as a cause of spinal cord compression
Connor Sweeney, Dennis Prangnell and David Wheatley
pp 9-11
Myeloid sarcoma is a rare solid tumour consisting of primitive myeloid cells that have infiltrated an extramedullary site. It is usually seen as a manifestation of acute myeloid leukaemia, but is occasionally associated with chronic myeloid leukaemia (CML) as well as myelodysplastic syndrome (MDS) and myeloproliferative disorders (MPDs). Although evidence of leukaemia is usually present in blood and bone marrow samples when the granulocytic sarcoma is discovered, the solid tumour can precede the detection of leukaemia.
Pregnancy in PNH: risks, mortality and monitoring
Abida Naeem, Anita Hill, Peter Hillmen and Richard J Kelly
pp 12-15
Paroxysmal nocturnal haemoglobinuria (PNH) is a rare, acquired disorder affecting haemopoietic stem cells (HSCs). Individuals with PNH develop haemopoietic clones which harbour somatic mutations of the phosphatidylinositol glycan complementation-class A gene (PIG-A). The PIG-A gene is needed for the synthesis of the glycophosphatidylinositol (GPI) anchor. Mutations of the PIG-A gene disrupt the GPI biosynthesis, leading to an absence or a reduction of the GPI anchor and, in turn, a marked deficiency of all GPI-linked proteins.

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