Myeloid & Lymphoid disorders in practice - 2011

Comment: Chronic myeloid leukaemia – 50 years of progress
John Reilly
pp 2-2
2010 witnessed the semicentennial of one of the most significant discoveries in the history of haematology, a finding that would revolutionise our understanding of malignancy and would lead to the first targeted therapy for cancer.

Chronic neutrophilic leukaemia: a rare diagnostic challenge
Youssef Sorour
pp 3-5
Chronic neutrophilic leukaemia (CNL) is a rare myeloproliferative neoplasm (MPN) characterised by sustained peripheral blood neutrophilia, bone marrow hypercellularity due to neutrophilic granulocyte proliferation, and hepatosplenomegaly. The diagnosis requires exclusion of reactive neutrophilia and other MPNs. CNL is universally negative for the Philadelphia (Ph) chromosome and the BCRABL1 fusion gene. To date, about 150 patients with this disorder have been reported.

New therapies in myelodysplastic syndromes
Sally Killick and David Bowen
pp 6-9
The myelodysplastic syndromes (MDS) are a diverse group of clonal haematological disorders. They are characterised by ineffective haematopoiesis leading to peripheral blood cytopenias and dysfunctional blood cells. They share the potential to progress to acute myeloid leukaemia (AML), the risk of which is related to the subtype.

Transient abnormal myelopoiesis in Down's syndrome infants
David KH Webb
pp 10-11
Children with Down’s syndrome are at risk of a variety of haematological disorders. The overall risk of leukaemia in Down's syndrome is increased ten- to 20-fold, and is especially marked in the first five years of life, when it is 50 times that in other children. The spectrum of myeloid disease includes a usually self-limiting myeloproliferative disorder of the newborn called transient abnormal myelopoiesis (TAM) or transient leukaemia, myelodysplastic syndromes (MDS), and acute myeloid leukaemia (AML).

An uncommon presentation of myelofibrosis
Moosa Qureshi and Claire N Harrison
pp 12-12
Myelofibrosis (MF) is a myeloproliferative neoplasm that typically exhibits clonal myeloproliferation, a leucoerythroblastic blood film with teardrop poikilocytes, bone marrow fibrosis, transfusion-dependent anaemia, constitutional symptoms and extramedullary haematopoiesis (EMH). The most common sites for EMH are the liver and spleen. Nonhepatosplenic EMH (NHS-EMH) is comparatively rare, diagnosed in only 5.3% of cases of EMH in a large retrospective analysis of 510 patients with EMH in 1975–2002 at Mayo Clinic. Cases of EMH that involved the lungs and/or pleura were exceedingly rare (0.6%). This case report illustrates the challenge of diagnosing and managing this uncommon presentation of MF.

Current UK trials for myeloproliferative neoplasms
Claire Woodley and Claire N Harrison
pp 13-15
Well-designed clinical trials and new therapies are key to advancing therapeutic strategies. How does this relate to the myeloproliferative neoplasms (MPNs)? In the UK, we have been successful with the Primary Thrombocythaemia-1 (PT-1) study and have also recently led and participated in the first JAK inhibitor study in Europe – COMFORT II. This study demonstrated superior results for INC424 (ruxolitinib) compared with physician’s choice of best available therapy, and will be presented at the American Society of Clinical Oncology and European Hematology Association (EHA) annual meetings in 2011.