Diagnosis and management of acquired aplastic anemia in childhood. Guidelines from the Marrow Failure Study Group of the Pediatric Haemato-Oncology Italian Association (AIEOP).

Acquired aplastic anemia (AA) is a rare heterogeneous disorder characterized by pancytopenia and hypoplastic bone marrow. The incidence is 2-3 per million population per year in the Western world, but 3 times higher in East Asia. Survival in severe aplastic anemia (SAA) has improved significantly due to advances in hematopoietic stem cell transplantation (HSCT), immunosuppressive therapy, biologic agents, and supportive care. In SAA, HSCT from a matched sibling donor (MSD) is the first-line treatment. If a MSD is not available, options include immunosuppressive therapy (IST), matched unrelated donor, or haploidentical HSCT. The purpose of this guideline is to provide health care professionals with clear guidance on the diagnosis and management of pediatric patients with AA. A preliminary evidence-based document prepared by a group of pediatric hematologists of the Bone Marrow Failure Study Group of the Italian Association of Pediatric Hemato-Oncology (AIEOP) was discussed, modified and approved during a series of consensus conferences that started online during COVID 19 and continued in the following years, according to procedures previously validated by the AIEOP Board of Directors.

Measurement of the Gamma-Ray-to-Neutron Branching Ratio for the Deuterium-Tritium Reaction in Magnetic Confinement Fusion Plasmas.

At present, magnetic confinement fusion devices rely solely on absolute neutron counting as a direct way of measuring fusion power. Absolute counting of deuterium-tritium gamma rays could provide the secondary neutron-independent technique required for the validation of scientific results and as a licensing tool for future power plants. However, this approach necessitates an accurate determination of the gamma-ray-to-neutron branching ratio. The gamma-ray-to-neutron branching ratio for the deuterium-tritium reaction ^{3}H(^{2}H,γ)^{5}He/^{3}H(^{2}H,n)^{4}He was determined in magnetic confinement fusion plasmas at the Joint European Torus in predominantly deuterium beam heated plasmas. The branching ratio was found to be equal to (2.4±0.5)×10^{-5} over the deuterium energy range of (80±20) keV. This accurate determination of the deuterium-tritium branching ratio paves the way for a direct and neutron-independent measurement of fusion power in magnetic confinement fusion reactors, based on the absolute counting of deuterium-tritium gamma rays.

Late-onset and long-lasting neutropenias in the young: A new entity anticipating immune-dysregulation disorders.

This study identifies a new chronic form of immune neutropenia in the young with or without detectable indirect anti-neutrophil antibodies, characterized by mild/moderate neutropenia low risk of severe infection (14%), tendency to develop autoimmune phenomena over the course of the disease (cumulative incidence of 58.6% after 20 years of disease duration), leukopenia, progressive reduction of absolute lymphocyte count and a T- and B-cell profile similar to autoimmune disorders like Sjogren syndrome, rheumatoid arthritis, and systemic lupus erythematosus (increased HLADR+ and CD3 + TCRγδ cells, reduced T regulatory cells, increased double-negative B and a tendency to reduced B memory cells). In a minority of patients, P/LP variants related to primary immuno-regulatory disorders were found. This new form may fit the group of "Likely acquired neutropenia," a provisional category included in the recent International Guidelines on Diagnosis and Management of Neutropenia of EHA and EUNET INNOCHRON ACTION 18233. The early recognition of this form of neutropenia would help clinicians to delineate better specific monitoring plans, genetic counseling, and potentially targeted therapies.