Nineteen patients with high‐risk myelodysplastic syndrome (MDS)/acute myeloid leukaemia (AML) received fludarabine, cytarabine, granulocyte‐colony stimulating factor (G‐CSF), and idarubicin chemotherapy (de novo MDS/MDS‐AML, nine; relapsed/refractory MDS/AML, seven; therapy‐related MDS, three). Median age was 44 years and median disease duration 10 months. 16/19 (84%) patients had abnormal cytogenetics with seven (37%) harbouring abnormalities of chromosome 7. 18/19 (94.7%) patients responded to FLAG‐idarubicin with 12 (63%) achieving complete remission (CR) (<5% blasts and normal cytogenetics). 7/9 (78%) patients with de novo MDS/MDS‐AML achieved CR compared to 5/10 (50%) with alternative diagnoses. Response was associated with age < 50 years, disease duration < 3 months, and cytogenetics other than abnormalities of chromosome 7. Haemopoietic regeneration was rapid in most patients and there were no toxic deaths. Nine patients received a second course of chemotherapy, three have proceeded to allogeneic bone marrow transplant and three to autologous blood stem cell/bone marrow transplantation. Follow‐up is short (median 10 months). 12/19 (63%) patients remain alive and 5/12 (42%) have relapsed at a median 5 months following CR achievement. FLAG‐idarubicin was well tolerated. High rates of morphological and cytogenetic remission, especially in de novo MDS, offer a window of opportunity for assessment of autologous BMT in this group of diseases where no treatment except alloBMT has led to prolongation of survival.

CTLAAla 17 ) and that this results in inefficient glycosylation and decreased cell-surface expression (Anjos et al., 2002). Our association results show that rs231775 was also strongly associated with AA in our sample although the P-values were less significant and the ORs were lower than those for rs3087243. Furthermore, conditional analysis revealed that rs1427678 explained the entire association signal at the locus.In conclusion, our results provide strong support for the hypothesis that CTLA4 is a susceptibility gene for AA, and they also suggest that it has the strongest effect in patients with a severe form of the disorder. Given the low P-values observed in our study and the genome-wide association study by Petukhova et al. (2010), we consider CTLA4 a proven susceptibility gene for AA.

Warfarin reversal is frequently required in day to day haematology practice. Prothrombin complex concentrates (PCC) have replaced fresh frozen plasma as the agent of choice in warfarin reversal due to its safety profile. Ideal dose of PCC is still not known and many centres have local guidelines on using the product. We have successfully used PCC (Beriplex) at a fixed dose in our hospital, but we still need randomised control trials to identify the ideal dose of PCC which can result in rapid reversal of warfarin with minimum risk of thrombosis.

Summary High oxygen affinity haemoglobins result in polycythaemia and cardiovascular adaptation to maintain tissue oxygenation. The polycythaemia can cause symptoms of hyperviscosity and vaso‐occlusive disease. We report a kindred with a high affinity haemoglobin (Haemoblobin Yakima) one of whose members gave birth to two infants with intra‐uterine growth retardation and who suffered with symptoms of hyperviscosity which settled on reduction of the PCV by venesection.

A young woman presented with a 2 year history of a severe bleeding disorder and marked deficiencies in all four vitamin-K-dependent factors. Metabolic studies with tracer doses of tritium-labelled vitamin K1 suggested that the patient might be taking an oral anticoagulant; and subsequently her plasma was found to contain a substance identical to phenindione in its spectrophotometric and chromatographic properties. The half-disappearance times of factors II, IX, X were measured after the administration of a concentrate of these factors and were found to conform with published figures. The concentrate controlled the patient's excessive bruising and prolonged skin and gingival bleeding. It would therefore seem that factor VII may not be essential in reversal of the bleeding disorder induced by anticoagulant overdose.