Severe lympho-depletion, abrogated thymopoiesis and systemic EBV positive T-cell lymphoma of childhood, a case.

Epstein-Barr virus (EBV) associated T-cell and NK-cell lymphoproliferative diseases are lethal and extremely rare in Caucasians. We expand on the clinical, immunological and histogenetic characteristics associated with this second European case (19 years old, previously healthy, Caucasian boy) of systemic EBV positive T-cell lymphoma of childhood. We report, as novel findings, severe lympho-depletion and abrogation of thymopoiesis secondary to severe EBV activation and excessive immune activation. Similar to the first European case, we also detected a somatic missense variant in the proto-oncogene FYN. In the first European patient however, the FYN variant allele frequency (VAF) was 10% and the patient only experienced moderate leukopenia, whereas in our case, the VAF was 48% and the patient experienced severe leukopenia and lymphopenia. This could suggest a pathogenic role of these FYN variants in driving excessive T cell activation. If confirmed, FYN might become target in future treatments of this fatal disorder.

BQ.1.1, XBB.1, and XBB.1.5 neutralization after bivalent mRNA COVID-19 booster in patients with cancer.

Ehmsen et al. evaluate the neutralizing capacity to current SARS-CoV-2 variants in patients with cancer before and after receiving the BNT162b2 bivalent mRNA vaccine booster. Bivalent vaccine provides some protection against BQ.1.1 but fails to protect against XBB.1 and XBB.1.5 in patients with cancer.

Repeatability and inter-observer variation of choroidal thickness measurements using swept-source optical coherence tomography in myopic danish children aged 6-14 years.

PURPOSE: To estimate repeatability and inter-observer variation of choroidal thickness measurements in myopic Danish children aged 7-14 years using swept-source optical coherence tomography (SS-OCT). METHODS: Thirty-nine children were enrolled. Optical correction was single-vision spectacles (SVS) or orthokeratology lenses (OKL). Three repeated 7 × 7 mm 3-dimensional SS-OCT macula scans and three repeated 9 mm SS-OCT line scans were collected for each child using a DRI OCT Triton. Choroidal thickness was measured using three different methods: line scan method 1 (LM1), line scan method 2 (LM2) and 3D macula scan method (3DM). Segmentation was adjusted if needed. Coefficients of repeatability (CR) and limits of agreement (LoA) were calculated. RESULTS: The CRs ranged from 13.4 to 23.9 µm, 14.5 to 26.2 µm and 5.2 to 10.7 µm for LM1, LM2 and 3DM, respectively. The LoA ranged from -22.9 to +31.5 µm, -23.3 to +32.2 µm and -10.2 to +12.4 µm for LM1, LM2 and 3DM, respectively. Segmentation was adjusted in most scans (63%-92%). Mean choroidal thickness ranged from 142.2 ± 47.2 to 253.8 ± 60.9 µm and 190.1 ± 64.0 to 299.0 ± 55.8 µm for the SVS and OKL groups, respectively, measured by 3DM. CONCLUSION: The 3DM was the most repeatable method in this paediatric population. It yielded a CR of 10.7 µm, confidence interval 2.4 µm, which makes the minimal detectable difference between two measurements 13.1 µm. Most inter-observer variation could be explained by the intramethod variation. Segmentation adjustment on 3D macula scans did not increase CR on a group level.