Clinicopathologic and genetic profile of intracranial marginal zone lymphoma: a primary low-grade CNS lymphoma that mimics meningioma.

PURPOSE: Although rare overall, marginal zone B-cell lymphoma (MZBCL) is the most common primary low-grade CNS lymphoma reported in the literature. The aim of this study is to elucidate the biology and genetic features of this unusual tumor. PATIENTS AND METHODS: Fifteen CNS MZBCLs were studied clinically, pathologically, and genetically, including fluorescent in situ hybridization analyses with commercially available MALT1 and IgH break-apart and centromere 3, 7, 12, and 18 probes. RESULTS: CNS MZBCLs preferentially affect middle-aged women (female-to-male ratio, 4:1), with 93% presenting as dural-based masses mimicking meningioma. Ten patients with 1 to 7.6 years of follow-up after diagnosis showed no evidence of disease after radiation and/or chemotherapy. Like MZBCLs outside of the CNS, they consisted of CD20+, CD3- small B lymphocytes with varying degrees of plasmacytic differentiation and predominantly kappa light-chain restriction (78%). Lymphoid follicles with follicular colonization were seen in three patients and deposition of amyloid was noted in samples from two patients, one of which was tumefactive. Neither Bcl-6 protein nor Epstein-Barr virus-encoded RNA was expressed. Trisomy 3 was detected in six of 12 patients, with no rearrangements of MALT1 or IgH and no trisomies of 7, 12, or 18 detected. CONCLUSION: Our data suggest that intracranial MZBCL is an indolent primary CNS lymphoma that typically presents as a meningioma-like dural-based mass. Trisomy 3, but not MALT1 or IgH translocation, is a common genetic abnormality that may contribute to the pathogenesis of this CNS lymphoma.     

An ATP-activated channel is involved in mitogenic stimulation of human T lymphocytes

We investigated the effect of pharmacologic modulation of the ATP receptor on intracellular ion changes and proliferative response of human peripheral blood lymphocytes (PBLs) and purified T lymphocytes. Extracellular ATP (ATPe) triggered in these cells an increase in the cytoplasmic Ca2+ concentration ([Ca2+]i) and plasma membrane depolarization. Whereas both Ca2+ release from intracellular stores and influx across the plasma membrane were detected in the whole PBL population, only Ca2+ influx was observed in T cells. In the presence of near physiologic extracellular Na+ concentrations (125 mmol/L), Ca2+ permeability through the ATPe-gated channel was very low, suggesting a higher selectivity for monovalent over divalent cations. The selective P2Z agonist benzoylbenzoic ATP (BzATP) increased [Ca2+]i in the presence but not the absence of extracellular Ca2+ and also caused plasma membrane depolarization. The covalent blocker oxidized ATP (oATP), an inhibitor of P2X and P2Z receptors, prevented Ca2+ influx and plasma membrane depolarization, but had no effect on Ca2+ release from stores. Stimulation with ATPe alone had no significant effects on PBL 3H-thymidine incorporation. On the contrary, ATPe or BzATP had a synergistic effect on DNA synthesis stimulated by selective T-cell mitogens such as phytohemagglutinin, anti-CD3 monoclonal antibody, or allogenic PBLs (mixed lymphocyte cultures). Treatment with oATP inhibited mitogenic stimulation by these receptor-directed agents but not by the combined application of the Ca2+ ionophore ionomycin and phorbol myristate acetate. Interleukin-2 partially relieved inhibition by oATP. These results suggest that human T lymphocytes express a plasma membrane channel gated by ATPe that is involved in mitogenic stimulation.     

Edward Zigler's legacy in the study of persons with intellectual disability: the developmental approach and the advent of a more rigorous and compassionate science

Edward Zigler transformed the science and humanity of the work with persons with intellectual disability. The developmental approach is Ed's great contribution to the field of intellectual disability as it both led to more conceptually compelling and methodologically rigorous science and provided an alternative to the Zeitgeist of segregation, defect, and pathology that had prevailed for decades. In an entirely unique way, the developmental approach allowed a seamless integration of increasingly precise science with concern for the “whole child” and their family. Thus, Ed's legacy led to a discipline in which scholarship and compassion prevail hand in hand as the integrity of science and of the person are mutually informative and interdependent.     

Combined endovascular and open repair of a penetrating innominate artery and tracheal injury

Endovascular therapy affords the opportunity to decrease surgical morbidity and improve operative planning in complex penetrating injuries of the chest. In this case report we describe a hemodynamically stable patient with a single gunshot wound to the base of the neck (zone I), with combined vascular and tracheal injuries. We present a novel approach to the repair of this type of injury using combined endovascular and open techniques.     

Perceptual processing among high-functioning persons with autism

Two tasks were used to assess the processing of whole versus parts of objects in a group of high-functioning children and adolescents with autism (N = 11) and a comparison group of typically developing peers (N = 11) matched for chronological age and IQ. In the first task, only the children with autism showed a global advantage, and the two groups showed similar interference between levels. In the second task, the children with autism, despite longer RTs, showed similar performance to the comparison group with regard to the effect of goodness on visual parsing. Contrary to expectations based on the central coherence and hierarchisation deficit theories, these findings indicate intact holistic processing among persons with autism. The implications of these findings are discussed in relation to apparently discrepant evidence from other studies.