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The study of (anti-)deuteron production in pp collisions has proven to be a powerful tool to investigate the formation mechanism of loosely bound states in high energy hadronic collisions. In this paper the production of (anti-)deuterons is studied as a function of the charged particle multiplicity in inelastic pp collisions at s√=13 TeV using the ALICE experiment. Thanks to the large number of accumulated minimum bias events, it has been possible to measure (anti-)deuteron production in pp collisions up to the same charged particle multiplicity (dNch/dη∼26) as measured in p-Pb collisions at similar centre-of-mass energies. Within the uncertainties, the deuteron yield in pp collisions resembles the one in p-Pb interactions, suggesting a common formation mechanism behind the production of light nuclei in hadronic interactions. In this context the measurements are compared with the expectations of coalescence and Statistical Hadronisation Models (SHM).
The first measurement at the LHC of charge-dependent directed flow (v1) relative to the spectator plane is presented for Pb-Pb collisions at sNN−−−√ = 5.02 TeV. Results are reported for charged hadrons and D0 mesons for the transverse momentum intervals pT>0.2 GeV/c and 3<pT< 6 GeV/c in the 5-40% and 10-40% centrality classes, respectively. The difference between the positively and negatively charged hadron v1 has a positive slope as a function of pseudorapidity η, dΔv1/dη=[1.68 ± 0.49 (stat.) ± 0.41 (syst.)] ×10−4. The same measurement for D0 and D¯0 mesons yields a positive value dΔv1/dη= [4.9 ± 1.7 (stat.) ± 0.6 (syst.)]×10−1, which is about three orders of magnitude larger than the one of the charged hadrons. These measurements can provide new insights into the effects of the strong electromagnetic field and the initial tilt of matter created in non-central heavy-ion collisions on the dynamics of light (u, d, and s) and heavy (c) quarks. The large difference between the observed Δv1 of charged hadrons and D0 mesons may reflect different sensitivity of the charm and light quarks to the early time dynamics of a heavy-ion collision. These observations challenge some of the recent theoretical calculations, which predicted a negative and an order of magnitude smaller value of dΔv1/dη for both light-flavour and charmed hadrons.
The production of K∗(892)0 and ϕ(1020) in pp collisions at s√ = 8 TeV was measured using Run 1 data collected by the ALICE collaboration at the LHC. The pT-differential yields d2N/dydpT in the range 0<pT<20 GeV/c for K∗0 and 0.4<pT<16 GeV/c for ϕ have been measured at midrapidity, |y|<0.5. Moreover, improved measurements of the K∗(892)0 and ϕ(1020) at s√=7TeV are presented. The collision energy dependence of pT distributions, pT-integrated yields and particle ratios in inelastic pp collisions are examined. The results are also compared with different collision systems. The values of the particle ratios are found to be similar to those measured at other LHC energies. In pp collisions a hardening of the particle spectra is observed with increasing energy, but at the same time it is also observed that the relative particle abundances are independent of the collision energy. The pT-differential yields of K∗0 and ϕ in pp collisions at s√=8 TeV are compared with the expectations of different Monte Carlo event generators.
The global polarization of the Λ and Λ¯¯¯¯ hyperons is measured for Pb-Pb collisions at sNN−−−√ = 2.76 and 5.02 TeV recorded with the ALICE at the LHC. The results are reported differentially as a function of collision centrality and hyperon's transverse momentum (pT) for the range of centrality 5-50%, 0.5<pT<5 GeV/c, and rapidity |y|<0.5. The hyperon global polarization averaged for Pb-Pb collisions at sNN−−−√ = 2.76 and 5.02 TeV is found to be consistent with zero, ⟨PH⟩ (%) ≈ - 0.01 ± 0.05 (stat.) ± 0.03 (syst.) in the collision centrality range 15-50%, where the largest signal is expected. The results are compatible with expectations based on an extrapolation from measurements at lower collision energies at RHIC, hydrodynamical model calculations, and empirical estimates based on collision energy dependence of directed flow, all of which predict the global polarization values at LHC energies of the order of 0.01%.
Chronic granulomatous disease (CGD) is a primary immunodeficiency, which is diagnosed in most patients between one and three years of age. Here we report on a boy who presented at birth with extensive skin lesions and lymphadenopathy which were caused by CGD. An analysis of the literature revealed 24 patients with CGD who became symptomatic during the first six weeks of life. Although pulmonary complications and skin lesions due to infection were the leading symptoms, clinical features were extremely heterogenous. As follow-up was not well specified in most patients, the long-term prognosis of children with very early onset of CGD remains unknown.
Survival following relapse in children with Acute Myeloid leukemia: a report from AML-BFM and COG
(2021)
Simple Summary: Acute myeloid leukemia in children remains a difficult disease to cure despite intensive therapies that push the limits of tolerability. Though the intent of initial therapy should be the prevention of relapse, about 30% of all patients experience a relapse. Hence, relapse therapy remains critically important for survival. This retrospective analysis of two large international study groups (COG and BFM) was undertaken to describe the current survival, response rates and clinical features that predict outcomes. We demonstrate that children with relapsed AML may be cured with cytotoxic therapy followed by HSCT. High-risk features at initial diagnosis and early relapse remain prognostic for post-relapse survival. Current response criteria are not aligned with the standards of care for children, nor are the count recovery thresholds meaningful for prognosis in children with relapsed AML. Our data provide a new baseline for future treatment planning and will allow an updated stratification in upcoming studies.
Abstract: Post-relapse therapy remains critical for survival in children with acute myeloid leukemia (AML). We evaluated survival, response and prognostic variables following relapse in independent cooperative group studies conducted by COG and the population-based AML-BFM study group. BFM included 197 patients who relapsed after closure of the last I-BFM relapse trial until 2017, while COG included 852 patients who relapsed on the last Phase 3 trials (AAML0531, AAML1031). Overall survival at 5 years (OS) was 42 ± 4% (BFM) and 35 ± 2% (COG). Initial high-risk features (BFM 32 ± 6%, COG 26 ± 4%) and short time to relapse (BFM 29 ± 4%, COG 25 ± 2%) predicted diminished survival. In the BFM dataset, there was no difference in OS for patients who had a complete remission with full hematopoietic recovery (CR) following post-relapse re-induction compared to those with partial neutrophil and platelet recovery (CRp and CRi) only (52 ± 7% vs. 63 ± 10%, p = 0.39). Among 90 patients alive at last follow-up, 87 had received a post-relapse hematopoietic stem cell transplant (HSCT). OS for patients with post-relapse HSCT was 54 ± 4%. In conclusion, initial high-risk features and early relapse remain prognostic. Response assessment with full hematopoietic recovery following initial relapse therapy does not predict survival. These data indicate the need for post-relapse risk stratification in future studies of relapse therapies.
Simple Summary: Children with acute myeloid leukemia (AML) experience high relapse rates of about 30%; still, survival rates following the first relapse are encouraging. Hence, it is critically important to examine the consequences of a second relapse; however, little is known about this subgroup of patients. This retrospective population-based analysis intends to describe response, survival and prognostic factors relevant for the survival of children with second relapse of AML. Treatment approaches include many different therapeutic regimens, including palliation and intensive treatment with curative intent (63% of the patients). Survival is poor; however, patients who respond to reinduction attempts can be rescued with subsequent hematopoietic stem cell transplantation. We deciphered risk factors, such as short time interval from first to second relapse below one year as being associated with a poor outcome. This analysis will help to improve future international treatment planning and patient care of children with advanced AML.
Abstract: Successful management of relapse is critical to improve outcomes of children with acute myeloid leukemia (AML). We evaluated response, survival and prognostic factors after a second relapse of AML. Among 1222 pediatric patients of the population-based AML-Berlin–Frankfurt–Munster (BFM) study group (2004 until 2017), 73 patients met the quality parameters for inclusion in this study. Central review of source documentation warranted the accuracy of reported data. Treatment approaches included palliation in 17 patients (23%), intensive therapy with curative intent (n = 46, 63%) and other regimens (n = 10). Twenty-five patients (35%) received hematopoietic stem cell transplantation (HSCT), 21 of whom (88%) had a prior HSCT. Survival was poor, with a five-year probability of overall survival (pOS) of 15 ± 4% and 31 ± 9% following HSCT (n = 25). Early second relapse (within one year after first relapse) was associated with dismal outcome (pOS 2 ± 2%, n = 44 vs. 33 ± 9%, n = 29; p < 0.0001). A third complete remission (CR) is required for survival: 31% (n = 14) of patients with intensive treatment achieved a third CR with a pOS of 36 ± 13%, while 28 patients (62%) were non-responders (pOS 7 ± 5%). In conclusion, survival is poor but possible, particularly after a late second relapse and an intensive chemotherapy followed by HSCT. This analysis provides a baseline for future treatment planning.
Given the ongoing global SARS-CoV-2-vaccination efforts, clinical awareness needs to be raised regarding the possibility of an increased incidence of SARS-CoV-2-vaccine-related immune-mediated thrombocytopenia in patients with intracerebral hemorrhage (ICH) secondary to cerebral sinus and vein thrombosis (CVT) requiring (emergency) neurosurgical treatment in the context of vaccine-induced immune thrombotic thrombocytopenia (VITT). Only recently, an association of vaccinations and cerebral sinus and vein thrombosis has been described. In a number of cases, neurosurgical treatment is warranted for these patients and special considerations are warranted when addressing the perioperative coagulation. We, herein, describe the past management of patients with VITT and established a literature-guided algorithm for the treatment of patients when addressing the impaired coagulation in these patients. Increasing insights addressing the pathophysiology of SARS-CoV-2-vaccine-related immune-mediated thrombocytopenia guide physicians in developing an interdisciplinary algorithm taking into account the special considerations of this disease.