Issue Archive

In this issue of Blood, Hahm and colleagues identify the extracellular protein disulfide isomerase (PDI) as an essential regulator of the adhesiveness of the β₂-integrin macrophage-1 antigen (Mac-1) on neutrophils.¹  In the absence of PDI, Mac-1–dependent neutrophil adhesion and crawling is reduced in vivo. Rescue experiments with exogenous PDI showed that the isomerase activity of extracellular PDI is critical for its regulatory effect on neutrophil recruitment. This intriguing finding suggests that disulfide bonds in Mac-1 regulate integrin activity and neutrophil recruitment.

In this issue of Blood, Bueno and colleagues explore the developmental impact, as well as the transforming capacity, of the mixed-lineage leukemia (MLL)–AF4 fusion protein in combination with activation of FMS-like tyrosine receptor 3 (FLT3) in human embryonic stem cells (hESCs).¹ 

In this issue of Blood, Apollonio et al report on a subset of chronic lymphocytic leukemia (CLL) characterized by molecular anergy and demonstrate how this phenotype could potentially be targeted therapeutically.¹ 

In this issue of Blood, Andolfo and colleagues show that dehydrated hereditary stomatocytosis (DHSt), an inherited red cell disorder, is associated with a number of distinct germline mutations in PIEZO1, a stretch activated cation channel, in 26 affected individuals from 7 families.¹ 

In this issue of Blood, Carcao et al demonstrate that the phenotypic variability in patients with severe hemophilia A correlates with the F8 mutation, where patients with non-null mutations exhibit a milder bleeding phenotype compared with those with null mutations, although this difference is not likely to influence the treatment decision making.¹ 

In this issue of Blood, Boelens et al report transplantation outcomes for the largest cohort assembled to date of patients with Hurler syndrome, demonstrating key associations with survival and outlining approaches that result in higher levels of α-l-iduronidase, the enzyme missing in this devastating disorder.¹