ABNORMALITIES IN THE RETINAL CAPILLARY PLEXUSES IN COATS DISEASE IN ADULTHOOD ON OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY.

PURPOSE: To describe and quantify the abnormalities of the retinal capillary plexuses using optical coherence tomography angiography in Coats disease. METHODS: Retrospective study. Eleven eyes of 11 patients with Coats disease (9 men and two women aged 32-80 years) compared with nine fellow eyes and 11 healthy control eyes. Horizontal bands of contiguous 3 × 3 mm optical coherence tomography angiograms of the superficial vascular plexus and deep capillary complex were acquired from the optic disk to 6 mm temporal to the fovea, through areas with telangiectasia visible on fluorescein angiography in 9 cases. RESULTS: The vascular density was significantly decreased in both plexuses in eyes with Coats disease compared with normal and fellow eyes within the 6 mm temporal to the fovea (superficial vascular plexus: 21.5 vs. 29.4%, P = 0.00004 and vs. 30.3%, P = 0.00008; deep capillary complex, 16.5 vs. 23.9%, P = 0.00004 and vs. 24.7%, P = 0.00008, respectively). The fractal dimension was also significantly decreased in eyes with Coats disease (superficial vascular plexus: 1.796 vs. 1.848 P = 0.001 and vs. 1.833, P = 0.003; deep capillary complex: 1.762 vs. 1.853, P = 0.003 and vs. 1.838, P = 0.004, respectively). CONCLUSION: Retinal plexuses' vascular density was decreased in Coats disease, including in areas with no visible telangiectasia.

Optical coherence tomography angiography analysis of changes in the retina and the choroid after hemodialysis for end stage kidney disease.

PURPOSE: To study effects of hemodialysis (HD) on retinal and choroidal vasculature in patients with end-stage kidney disease (ESKD) using optical coherence tomography angiography (OCTA). METHODS: In this prospective study, we investigated eyes of patients undergoing hemodialysis for ESKD from Mondor University Hospital. Only one eye/patient was considered. Subfoveal choroidal thickness (SCT) was measured on enhanced-depth imaging optical coherence tomography (EDI-OCT) before and after the hemodialysis session. OCTA was used to extract retinal vascular density (superficial and deep capillary plexus, SCP, DCP) and choriocapillaris non-perfusion. Clinical, demographic and biological parameters (Blood B-Nitric Peptid rate prior to HD session) were reviewed. RESULTS: Twenty patients (mean age 53.2 ± 13.6 years, 10 males and 10 females) were included in this prospective study. SCT significantly decreased after the HD session (234.3 ± 56.14 µm before HD to 211.9 ± 60.79 µm after hemodialysis (Wilcoxon signed-rank test, p = 0.003)). Non-perfusion in the choriocapillaris significantly increased after HD (41.65 ± 3.58 before HD, 42.95 ± 3.19 after HD, p = 0.036) while no significant modification of the vascular density was observed in the retinal vasculature (SCP, DCP) around the macular zone or the optic nerve. An increased plasma B-Nitric Peptide (BNP) level prior to the onset of the HD session was significantly correlated with the decrease of the SCT (r = 0.45, p = 0.043). CONCLUSION: Hemodialysis in patients with ESKD is associated with a significant decrease in SCT and an increase in non-perfusion in the choriocapillaris on OCTA. A High BNP level prior to the onset of the hemodialysis appears to be correlated with the decrease in SCT.

CD200/BTLA deletions in pediatric precursor B-cell acute lymphoblastic leukemia treated according to the EORTC-CLG 58951 protocol.

DNA copy number analysis has been instrumental for the identification of genetic alterations in B-cell precursor acute lymphoblastic leukemia. Notably, some of these genetic defects have been associated with poor treatment outcome and might be relevant for future risk stratification. In this study, we characterized recurrent deletions of CD200 and BTLA genes, mediated by recombination-activating genes, and used breakpoint-specific polymerase chain reaction assay to screen a cohort of 1154 cases of B-cell precursor acute lymphoblastic leukemia uniformly treated according to the EORTC-CLG 58951 protocol. CD200/BTLA deletions were identified in 56 of the patients (4.8%) and were associated with an inferior 8-year event free survival in this treatment protocol [70.2% ± 1.2% for patients with deletions versus 83.5% ± 6.4% for non-deleted cases (hazard ratio 2.02; 95% confidence interval 1.23-3.32; P=0.005)]. Genetically, CD200/BTLA deletions were strongly associated with ETV6-RUNX1-positive leukemias (P<0.0001), but were also identified in patients who did not have any genetic abnormality that is currently used for risk stratification. Within the latter population of patients, the presence of CD200/BTLA deletions was associated with inferior event-free survival and overall survival. Moreover, the multivariate Cox model indicated that these deletions had independent prognostic impact on event-free survival when adjusting for conventional risk criteria. All together, these findings further underscore the rationale for copy number profiling as an important tool for risk stratification in human B-cell precursor acute lymphoblastic leukemia. This trial was registered at www.ClinicalTrials.gov as #NCT00003728.