The human syndrome of dendritic cell, monocyte, B and natural killer lymphoid deficiency presents as a sporadic or autosomal dominant trait causing susceptibility to mycobacterial and other infections, predisposition to myelodysplasia and leukemia, and, in some cases, pulmonary alveolar proteinosis. Seeking a genetic cause, we sequenced the exomes of 4 unrelated persons, 3 with sporadic disease, looking for novel, heterozygous, and probably deleterious variants. A number of genes harbored novel variants in person, but only one gene, GATA2, was mutated in all 4 persons. Each person harbored a different mutation, but all were predicted to be highly deleterious and to cause loss or mutation of the C-terminal zinc finger domain. Because GATA2 is the only common mutated gene in 4 unrelated persons, it is highly probable to be the cause of dendritic cell, monocyte, B, and natural killer lymphoid deficiency. This disorder therefore constitutes a new genetic form of heritable immunodeficiency and leukemic transformation. (Blood. 2011;118(10):2656-2658)

Human immunodeficiency syndrome with loss of DCs, monocytes, and T reg cells; preservation of Langerhans cells; associated loss of BM multilymphoid progenitors; and overproduction of Flt3 ligand.

No evidence has been found, analyzed by intention to treat, that use of WBC-reduced, BCD, or WCF RBCs reduces postoperative inpatient infection in patients undergoing cardiac bypass surgery.

TRALI is the most common serious adverse effect of blood transfusion in our hospital. Antibodies to HLA class II antigens should be looked for routinely when investigating a possible case of TRALI.

Our findings confirm the previous regional finding that the percentage of RBC units that are transfused to surgical patients in England and North Wales is now much lower than for medical patients and suggest that PBM initiatives should now focus on medical patients.

Falling donor numbers and the threat of transfusion-transmitted variant Creutzfeldt-Jakob disease may lead to shortages in the national blood supply. Knowledge of current patterns of transfusion and trends in usage will help predict future change in blood use. Our previous survey identified medical indications as the major reason for transfusion, but detailed information within this category was limited. We performed prospective surveys of indications for red cell transfusion for two 14-day periods in 2004 in the North of England, concentrating on medical reasons for transfusion. Data were obtained for transfusion indications of 9003 units, which accounted for over 99% of red cell issues from the regional blood centre during the study. In 2004, medical patients received 62% (5558 units) of all transfused units, surgical patients 33% (3001 units) and Obstetric & Gynaecology patients 5% (444 units). These figures compare with 52, 41 and 6% for Medicine, Surgery, and Obstetrics & Gynaecology in 1999/2000. The three largest uses of blood within the medical category were for patients with primary haematological disorders (18.2% of all transfused blood), for management of gastrointestinal haemorrhage (13.8%) and for patients with nonhaematological malignancies (8.8%). There has been a significant reduction in use of blood for surgical indications over the last 5 years, but an absolute increase in use of blood for medical indications. Lower transfusion triggers, education, use of cell salvage, the increasing price of a unit of red cells and changing population demographics may all have contributed to the reduction in surgical blood use. Promotion of good transfusion practice and alternatives to allogeneic transfusion should now focus on medical and surgical use of blood transfusion.

Posttransfusion survival is lower than estimated in previous decades in other countries. This is probably due to a relative increase in use of transfusion for older patients and for medical indications. Our figures may be used to predict and stratify the risk of infections, such as variant CJD, amongst different groups of transfusion recipients.

Further decreases in surgical RBC use may be achievable but the aging population is likely to demand more blood for nonsurgical indications and efforts should be directed to optimizing use in these recipients. Comparative data on transfusion rates between regions or countries may be a useful tool for improving blood use.