HLA frequency distribution of the Portuguese bone marrow donor registry.

Introduction: The Portuguese donor Registry of CEDACE was the fifth largest bone marrow donor Registry of the WMDA as of 2019 and has yet to be thoroughly analyzed. We aimed to characterize its various aspects, including demographics and HLA allele and haplotype frequencies, to evaluate the genetic matching propensity score and ultimately further develop it.

Methods: We described and compared characteristics of the donor population with census data and used an Expectation-Maximization algorithm and analyses of molecular variance to assess haplotype frequencies and establish phylogenetic distances between regions and districts within the country.

Chronic lymphocytic leukaemia/small lymphocytic lymphoma treatment with rituximab and high-dose methylprednisolone, revisited.

High-dose methylprednisolone plus rituximab (R-HDMP) is a useful treatment in chronic lymphocytic leukaemia/small lymphocytic lymphoma (CLL/SLL) patients unfit for chemo-immunotherapy and has proven its utility on the treatment of CLL/SLL complicated by auto-immune cytopenias. We performed a retrospective, single-centre study, of CLL/SLL patients treated with R-HDMP for 9 years. Thirty-nine patients were included, median age at time of treatment was 77 years.

Genetic Variation in PFKFB3 Impairs Antifungal Immunometabolic Responses and Predisposes to Invasive Pulmonary Aspergillosis.

Activation of immune cells in response to fungal infection involves the reprogramming of their cellular metabolism to support antimicrobial effector functions. Although metabolic pathways such as glycolysis are known to represent critical regulatory nodes in antifungal immunity, it remains undetermined whether these are differentially regulated at the interindividual level. In this study, we identify a key role for 6-phosphofructo-2-kinase/fructose-2,6-bisphosphatase 3 (PFKFB3) in the immunometabolic responses to Aspergillus fumigatus.

BK virus-specific T-cell immune reconstitution after allogeneic hematopoietic cell transplantation.

Clinical disease caused by BK virus reactivation is a frequent complication of allogeneic hematopoietic cell transplantation (HCT). Because of the lack of effective antiviral agents, BK virus-specific T cells are emerging as a potential therapy for BK virus disease, but the immune response to BK virus after allogeneic HCT has not been well characterized. Our study describes reconstitution of BK virus-specific T-cell immunity in 77 adult patients after HCT.

An activated Th17-prone T cell subset involved in chronic graft-versus-host disease sensitive to pharmacological inhibition.

Chronic graft-versus-host disease (cGvHD) remains a major complication of allogeneic stem cell transplantation requiring novel therapies. CD146 and CCR5 are expressed by activated T cells and associated with increased T cell migration capacity and Th17 polarization. We performed a multiparametric flow cytometry analysis in a cohort of 40 HSCT patients together with a cGvHD murine model to understand the role of CD146-expressing subsets.

Synchronous brain and intravascular B-cell lymphoma after remission of an adult hemophagocytic syndrome.

Hemophagocytic lymphohistiocytosis (HLH) should be considered in the differential diagnosis of adult patients with white matter disease. Brain involvement can be life-threatening and should prompt aggressive therapy. Even after HLH remission, the possibility of subsequent deterioration due to emergence of an aggressive intravascular lymphoma is highlighted here.