Acute annular outer retinopathy preceded by invasive ductal breast carcinoma: a case report.

BACKGROUND: Acute annular outer retinopathy (AAOR) is an uncommon disease. To date, there are few documented cases in the literature. Our case report is the first to describe a case of acute annular outer retinopathy in a patient with invasive ductal breast carcinoma. CASE PRESENTATION: The patient presented with photopsias and visual loss approximately 3 weeks prior to a diagnosis of invasive ductal breast carcinoma. We have documented the outer annular white ring seen in the acute phase of this disease and correlate it anatomically with Spectral-domain optical coherence tomography (SD-OCT) imaging. We identified RPE atrophy with nodular hyperreflectivity and loss of ellipsoid layer within the white annular ring with corresponding visual field loss. Fundus autofluorescence correlated with structural alterations seen on SD-OCT and showed both presumed active hyperautofluorescent zones with patchy hypoautofluorescent zones of atrophy and a classic annular hyperautofluorescent border. This case provides additional information about the natural history of this rare entity and its prognosis and varied presentation. CONCLUSIONS: The authors report a single case of acute annular outer retinopathy in a patient with invasive ductal breast carcinoma with the corresponding SD-OCT, fundus autofluorescence and visual field findings, during the acute phase of the disease. These findings provide new insight into the characteristic features, etiology and progression of this rare disease.

Management of pembrolizumab-induced steroid refractory mucositis with infliximab: A case report.

BACKGROUND: Pembrolizumab is an anti-programmed death receptor 1 (PD-1) that was shown to have a tolerable safety profile with 17% of grade 3-4 drug-related adverse events, notable response rate of 16% with median duration of response of 8 mo, and median overall survival of 8 mo. Severe mucositis is a very rare complication with only two cases of grade 4 mucositis reported, and both cases had good response to intravenous methylprednisolone and subsequent oral prednisone tapering. We report the first case of pembrolizumab-induced severe mucositis that was refractory to steroid treatment. CASE SUMMARY: An 80-year-old woman with a past medical history of recurrent right cheek nodular melanoma status post resection and new right lung metastatic melanoma on immunotherapy presented with dysphagia and odynophagia for 2 mo. She initially received 2 doses of ipilimumab 1 year ago with good outcome, but treatment was discontinued after developing severe diarrhea and rash. Pembrolizumab was then initiated 4 mo after disease progression. Significant improvement was noted after 3 doses. However, after 6 cycles of pembrolizumab, patient developed odynophagia and malnutrition. Improvement of symptoms was noted after discontinuation of pembrolizumab and initiation of steroids. 3 mo later, patient developed pharyngeal swelling with hoarseness and new oxygen requirement due to impending airway obstruction while being on prednisone tapering regimen, finally ended up with intubation and tracheostomy. Histologic analysis of left laryngeal and epiglottis tissue showed granulation tissue with acute on chronic inflammation, negative for malignancy and infection. Patient achieved marked improvement after 2 doses of infliximab of 5 mg/kg every 2 wk while continuing on prednisone tapering course. CONCLUSION: We report the first case of pembrolizumab-induced grade 4 mucositis that had limited recovery with prolonged steroid course but had rapid response with addition of infliximab. The patient had recurrent mucositis symptoms whenever steroids was tapered but achieved complete response after receiving two doses of infliximab while continuing to be on tapering steroids. The success of infliximab in this patient with pembrolizumab-induced severe mucositis presents a potentially safe approach to reduce prolonged steroid course and accelerate recovery in managing this rare complication.

Myroides spp. cellulitis and bacteremia: A case report.

Myroides spp., formerly Flavobacterium spp., are gram-negative, non-motile, traditionally opportunistic pathogens that are infrequent clinical isolates. Myroides spp. have been pathogenically implicated in only 52 reported cases since the discovery of the Flavobacterium genus in the 1920s. We present a case of Myroides spp. lower extremity cellulitis and bacteremia. To our knowledge, this is the 16th case of cutaneous infection caused by Myroides spp. Etiology of this patient's infection was felt to be related to exposure of his pre-existing lower extremity wounds to soil and water versus a dog lick in the context of relative immunosuppression from type 2 diabetes and chronic inhaled steroid use. Given the characteristic multi-drug resistance of Myroides spp., resistance to usual empiric antimicrobials given for cellulitis, and potential for fatal infection in cases of pan-resistance, it is important that clinicians remain alert to the possibility of this rare pathogen.

Ten-year retrospective review of outcomes following phacoemulsification with intraocular lens implantation in patients with pre-existing uveitis.

OBJECTIVE: The aim of this study was to evaluate the visual outcome, uveitis control, and complications following cataract surgery for intraocular lens (IOL) implantation in patients with a known diagnosis of uveitis. DESIGN: The study was a retrospective interventional case series. PARTICIPANTS: We reviewed 98 patients (137 eyes) with adult uveitis undergoing cataract surgery with foldable acrylic posterior chamber IOL implantation between 2003 and 2013 in 2 uveitis practices. METHODS: Best-corrected visual acuity (BCVA) and uveitis grade (Standardized Uveitis Nomenclature criteria) were measured at 1 month preoperatively, at postoperative week 1, and at postoperative months 1, 6, and 12. The main outcome measures were mean change in postoperative BCVA, uveitis grade, and complications. RESULTS: Of the eyes studied, 84% had grade 0-0.5 anterior uveitis at postoperative week 1 and maintained uveitis control (77% grade 0; 19% grade 0.5 anterior uveitis) at 1 year postoperatively. None of the patients had active intermediate or posterior uveitis at any time point. Mean BCVA improved from 0.71 ± 0.38 logMAR preoperatively to 0.37 ± 0.36 at 6 months (p < 0.01) and to 0.30 ± 0.25 at 12 months (p = 0.01) postoperatively. Of the study participants, 30% had preoperative complications related to uveitis, including epiretinal membrane (12%), cystoid macular edema (12%), and glaucoma (5.8%); 46% of patients had small pupils as a result of posterior synechiae. Postoperative vision-limiting complications included posterior capsule opacification (18%), epiretinal membrane (9.0%), and cystoid macular edema (8.8%). Of the eyes studied, 5.8% underwent Nd:YAG capsulotomy. CONCLUSIONS: Cataract surgery with acrylic posterior-chamber IOL implantation is effective at improving visual acuity in patients with uveitis. Uveitis was well controlled in the majority of our study patients for 12 months after cataract surgery. The most frequent vision-limiting postoperative complication was posterior capsule opacification, which was treatable with Nd:YAG capsulotomy.

CD34 expression predicts an adverse outcome in patients with NPM1-positive acute myeloid leukemia.

Patients with acute myeloid leukemia (AML) harboring an NPM1 mutation exhibit a heterogeneous clinical outcome. Recent studies have shown that the absence of FLT3 internal tandem duplication (FLT3-ITD) mutation confers a favorable prognosis in NPM1-positive AML. However, the prognostic impact of immunophenotypes in this subgroup remains unclear. In this study, FLT3 mutation status and immunophenotypic profile of 85 NPM1-positive patients with de novo AML were retrospectively analyzed and correlated with their clinical features and survival outcomes. Univariate analysis detected 5 markers with prognostic relevance: older age (≥60 years), high white blood cell (WBC) count (>30 × 10(9)/L), FLT3-ITD, CD7, and CD34 expression. Multivariate analysis showed that high WBC count was the only independent predictor of a lower complete remission rate (P = .019). Older age (P = .035), high WBC count (P = .008), FLT3-ITD (P = .012), and CD34 expression (P = .006) were independent predictors of a shorter event-free survival (EFS). High WBC count (P = .014), FLT3-ITD (P = .005), and CD34 expression (P = .047) were independent predictors of a shorter overall survival (OS). Furthermore, based on FLT3-ITD status in NPM1 mutation-positive patients, we showed that both high WBC and CD34 expression conferred a poor EFS (P = .010 and P = .016, respectively) and OS (P = .032 and P = .001, respectively) in the FLT3-ITD-negative group, whereas high WBC predicted a poor EFS (P = .016) and OS (P = .027) in the FLT3-ITD-positive group. Our results confirm the prognostic value of assessing FLT3-ITD mutations in NPM1-positive AML and identify the adverse prognostic impact of high WBC and CD34 expression in this subgroup of AML.

Prognostic value of immunophenotyping and gene mutations in elderly patients with acute myeloid leukemia with normal karyotype.

Elderly patients with acute myeloid leukemia generally have a poor prognosis and a highly heterogeneous clinical outcome. Prognostic indicators are required for and aid in patient stratification. However, the prognostic value of genetic mutations and immunophenotypic features in elderly normal karyotype acute myeloid leukemia, the largest cytogenetic risk group, remains unclear. We investigated the genetic mutations NPM1, FLT3-ITD, and FLT3-TKD and expression of the membrane antigens CD7, CD15, CD34, and CD56 in 144 elderly patients with de novo normal karyotype acute myeloid leukemia to retrospectively analyze the prognostic and clinical relevance of these parameters. CD7, CD15, CD34, and CD56 were expressed in 24%, 47%, 52%, and 15% of patients, respectively. NPM1 and FLT3-ITD mutations were detected in 51% and 17% of patients, respectively. Complete remission was obtained in 94 patients (65%), and the median overall survival was 16.5 months. Univariate analysis detected 5 markers with prognostic relevance: high leukocyte count, FLT3-ITD mutations, NPM1 mutations, CD34 expression, and CD56 expression in acute myeloid leukemia blasts. In multivariate analysis, patients with NPM1 predicted a higher complete remission (CR) rate (P = .016), longer event-free survival (P = .008), and longer overall survival (P = .049). FLT3-ITD mutations predicted a shorter event-free survival (P = .002) and shorter overall survival (P < .001). CD56 remained an independent predictor for lower CR rate (P = .021) and shorter event-free survival (P = .002). Our data highlight the prognostic importance of both genetic and immunophenotypic characteristics in this population of elderly patients with newly diagnosed normal karyotype acute myeloid leukemia. By combining genetic and immunophenotypic markers, we can divide patients into distinct prognostic groups with important implications for prognostic stratification and risk-adapted therapy.