Orbital diffuse large B-cell lymphoma with combined variable immunodeficiency.

Common variable immunodeficiency (CVID) is a primary immunodeficiency manifesting as a reduction in the level of total immunoglobulin (Ig) G, a reduction in the level of either IgA or IgM, poor response to polysaccharide vaccine, and usually frequent infections. The association of CVID with an increased risk of malignancy, specifically lymphoma, is well known. A 63-year-old female with a past medical history significant for CVID presented with a 1-month history of dull, left eye pain with proptosis, hypoglobus, and left upper lid fullness without a discrete palpable mass. Magnetic resonance imaging (MRI) of the orbits revealed a diffuse infiltrating orbital mass superonasally with extension into the superior rectus muscle, medial rectus muscle, and optic nerve up to the orbital apex and ethmoid sinus. A superonasal orbital biopsy with a caruncular approach was performed and demonstrated a sparse lymphoid infiltrate that was suggestive for a large B-cell neoplasm. Positron emission tomography (PET) scan demonstrated a hypermetabolic right lymph node, anterior to the right submandibular gland, which was biopsied and histopathology confirmed diffuse large B-cell lymphoma (DLBCL). Our patient achieved a very good response to chemotherapy with minimal residual disease on PET scan at the end of treatment. She attained a complete remission after radiation therapy. In conclusion, patients with new orbital and adnexa masses in the setting of a primary immunodeficiency can have an aggressive malignancy such as DLBCL and early diagnosis and systemic treatment carries a good prognosis.

Comparison of anti-VEGF therapies on fibrovascular pigment epithelial detachments in age-related macular degeneration.

BACKGROUND: The aim is to compare the therapeutic effects of three antivascular endothelial growth factor (VEGF) drugs (bevacizumab, aflibercept and ranibizumab) on fibrovascular pigment epithelial detachments (fvPEDs) in age-related macular degeneration (AMD). METHODS: This was a retrospective, comparative, consecutive case series of 88 unique eyes with fvPEDs in neovascular AMD treated with anti-VEGF monotherapy for a minimum of 6 months. All eyes were treatment naive. Diagnosis was confirmed retrospectively by fluorescein angiography and spectral-domain optical coherence tomography. Exclusion criteria included serous/drusenoid PEDs or patients who switched anti-VEGF. Mean follow-up across all therapies was 313.9±85.3 days. RESULTS: Average age of all patients was 80.6 years. Baseline maximum subfoveal PED height was 326.8±185.1 µm, 394.5±238.6 µm and 258.0±145.3 µm for bevacizumab, aflibercept and ranibizumab, respectively (p=0.05). All patients had subretinal fluid, intraretinal fluid or a combination of the two at an initial presentation. Central retinal thickness decreased at all time points compared with baseline across all three anti-VEGF therapies. Subfoveal PED height decreased in patients treated with aflibercept at all time points and decreased in patients treated with bevacizumab at 1-month, 3-month and 6-month time points. Aflibercept reduced PED height more than bevacizumab at 1-month and 12-month follow-ups (p=0.02 and p=0.03, respectively) and ranibizumab at 1-month and 6-month follow-ups (p=0.03 and p=0.02, respectively). No differences in best-corrected visual acuity were appreciated at any time point between drugs. CONCLUSIONS: There was a significant reduction in subfoveal PED height for aflibercept and bevacizumab compared with baseline. A direct comparison of drugs demonstrated a beneficial reduction of PED height, albeit inconsistently, favouring aflibercept. There were no differences in visual acuity across the groups at any time point.

VOLUMETRIC SINGLE-LAYER INNER RETINAL ANALYSIS IN PATIENTS WITH HYDROXYCHLOROQUINE TOXICITY.

PURPOSE: To compare retinal layer volumes using spectral-domain optical coherence tomography between eyes with hydroxychloroquine (HCQ) toxicity and control eyes. METHODS: Using a previously validated algorithm, volumetric analysis from the macular cube scan of the ganglion cell layer, inner plexiform layer, inner nuclear layer, and outer retina (outer plexiform layer to retinal pigment epithelium) layers were compared in three sets of patients: patients with a clinical diagnosis of HCQ toxicity, age-matched patients taking HCQ but not manifesting overt toxicity, and age-matched control patients. RESULTS: There were 14 patients in each group. The ganglion cell layer (P = 0.01), inner plexiform layer (P = 0.004), inner nuclear layer (P < 0.001), and outer plexiform layer to retinal pigment epithelium (P < 0.001) volumes were significantly reduced in HCQ toxicity eyes relative to the HCQ exposure eyes. There were no significant inner and outer retinal volume differences between the HCQ exposure group and group with no HCQ use (P > 0.05 for all layers). Increasing disease severity correlated with increasing volume loss in the inner retina (2.27 mm in early disease vs. 1.78 mm in advanced retinopathy, P = 0.02). CONCLUSION: Hydroxychloroquine toxicity seems to result in both outer and inner retinal volumetric thinning compared with age-matched control patients and patients taking HCQ but not manifesting toxicity.

Quantitative cerebral MR perfusion imaging: preliminary results in stroke.

PURPOSE: To evaluate quantitative cerebral blood flow (qCBF) with traditional time-based measurements or metrics of cerebral perfusion: time to peak (Tmax) and mean transit time (MTT) in stroke patients. MATERIALS AND METHODS: Nine ischemic stroke patients (four male, five female, 63 ± 16 years old) were included in the study which was Health Insurance Portability and Accountability Act compliant and institutional review board approved. Cerebral perfusion was quantified using the Bookend method. Mean values of qCBF, Tmax, and MTT were determined in regions of interest (ROIs). ROIs were drawn on diffusion weighted images in diffusion positive, critically ischemic (CI), in ipsilateral normal region immediately surrounding the critically ischemic region, the presumed penumbra (PP), and in contralateral diffusion negative control, presumed normal region (PN) of gray and white matter separately (GM and WM). RESULTS: In both GM and WM, qCBF measures distinguished the studied brain regions with the most markedly reduced values in regions corresponding to extent of likely ischemic injury. In planned comparisons, only qCBF measurements differed significantly between CI and PP tissues. ROC analysis supported the utility of qCBF for discriminating brain regions differing in the likely extent of ischemic injury (CI and PN regions - qCBF: area under the curve [AUC] = 0.96, Tmax: AUC = 0.96, MTT: AUC = 0.72). Importantly, qCBF afforded the best discrimination of CI and PP regions (qCBF: AUC = 0.82, Tmax: AUC = 0.65, MTT: AUC = 0.52). CONCLUSION: This initial evaluation indicates that quantitative MRI perfusion is feasible in ischemic stroke patients. qCBF derived with this strategy provide enhanced discrimination of CI and PP compared to time-based imaging metrics. This approach merits investigation in larger clinical studies.