Allergic bronchopulmonary aspergillosis (ABPA) is an eosinophilic pulmonary disorder caused by a hypersensitivity reaction to Aspergillus fumigatus that manifests with uncontrolled asthma, peripheral blood eosinophilia, and radiological findings, such as mucus plugging. Early diagnosis and proper treatment of ABPA are essential to prevent irreversible lung damage such as pulmonary fibrosis and bronchiectasis and improve the quality of life of patients. Beside inhaled medication for asthma, anti-inflammatory agents (i.e., systemic glucocorticoids) and antifungal agents are the mainstay treatment of ABPA. The goal of therapy using glucocorticoids and antifungal agents is to suppress the immune hyperreactivity to A. fumigatus and attenuate the fungal burden. Since the systemic glucocorticoid therapy may lead to serious adverse effects including osteoporosis, avascular necrosis, myopathy, cushingoid appearance, hypertension, insomnia, and increased risk of infection, a glucocorticoid-sparing agent could be considered. Mepolizumab is a humanized monoclonal antibody that binds to interleukin-5, which is the key mediator for eosinophil differentiation, activation, migration, and survival. We review eight cases of ABPA treated successfully with mepolizumab. Treatment with mepolizumab was not restricted to the total immunoglobulin E level, the limiting factor for omalizumab in ABPA. In addition, mepolizumab therapy improved forced expiratory volume in one second, radiological findings, and patient quality of life.
Rosai-Dorfman disease (RDD) is a rare medical condition with bilateral painless lymphadenopathy. We present the case of a young man diagnosed with a very unique presentation of Rosai-Dorfman disease.A 40-year-old African-American man presented with a firm, non-tender, progressive chest and neck mass appeared three months ago. Imaging of the neck demonstrated an 8.6-cm anterior neck subcutaneous soft tissue mass extending into the anterior mediastinum through the sternum with erosive changes in the sternum and the lesion is abutting the right common carotid artery and innominate vein and surrounds the medial aspect of the clavicles bilaterally. Ultrasound (US)-guided biopsy showed marked polytypic-appearing plasma cell proliferation associated with relatively prominent histiocytes with hemophagocytosis/emperipolesis and focal neutrophils. There were S100+ histiocytes; however, findings were not typical for RDD. As that biopsy was not diagnostic, incisional biopsy with adequate sampling was performed. Surgical pathology demonstrated a very abnormal infiltrate with prominent histiocytes including areas with the features of extranodal RDD. BRAF V600E immunohistochemistry (IHC) was negative. Modified radical neck dissection, proximal sternal resection and superior mediastinal nodal dissection surgery was recommended. However, the patient refused the procedure.
We present a case of a 58-year-old man with delayed diagnosed moyamoya disease who underwent encephaloduroarteriosynangiosis (EDAS) procedure. This patient with a history of three strokes presented to our facility with new left facial droop. Neurological examination revealed left facial droop and hemiparesis. Brain magnetic resonance imaging (MRI) described right frontal lobe acute ischemia. Head computed tomography (CT) angiography revealed bilateral supraclinoid internal carotid artery (ICA) occlusions. Cerebral angiography demonstrated diffuse intracranial vascular irregularity with stenosis, more above the bilateral supraclinoid ICAs and the right middle cerebral artery (MCA) suggestive of moyamoya disease. Due to the lack of MCA patency, he underwent EDAS. Superficial temporal artery (STA) was dissected inferiorly and the posterior branch was bipolared, then STA was movable. A bur hole made at the superior and inferior portion along the STA. Dura was opened, and STA was brought on top of the pia. His facial droop gradually improved after that. Nine months later, no new strokes reported.
Hypertrophic cardiomyopathy (HCM) and Wolff-Parkinson-White syndrome have been associated with sudden cardiac death. A subcutaneous implantable cardioverter-defibrillator (S-ICD) is an effective device used to reduce the risk of sudden cardiac death in these patients. The most common cause of inappropriate shocks with S-ICD is T-wave oversensing. We present the case of a 19-year-old man with repeated shocks from his S-ICD. This case highlights some of the sensing issues related to the S-ICD that can result in inappropriate shocks. A vector change may have occurred after T-wave remodeling, post accessory pathway ablation, and loss of R-waves due to HCM scar progression, leading to this consequence.
e12506 Background: Breast cancer accounts for a million new cases annually worldwide with 40,000 deaths reported in 2020 in the United States. Treatment includes surgery, adjuvant chemotherapy, radiotherapy and endocrine therapy. Oncotype recurrence score (RS), a 21-gene expression assay, is a tool to predict the benefit of adjuvant chemotherapy in hormone receptor (HR) positive breast cancer. High RS suggests benefit with chemotherapy. The goal of this study is to evaluate the relationship of the Oncotype RS with traditional prognostic/predictive factors. Methods: Retrospective IRB approved study of all patients from January 1, 2017, to December 31, 2019 with invasive breast cancer and an Oncotype RS in a community hospital setting. Study parameters included patient age, grade, tumor size, histologic subtype, HR status, number positive (0-4) lymph nodes (LN), extra nodal extension (ENE), lymphovascular invasion (LVI), and RS. Statistical analysis was done using linear regression and one-way ANOVA followed up with Tukey’s procedure. Results: A total of 470 patient charts evaluated and 22 patients excluded due to incomplete data with the following breakdown. LN positive = 14% (61/450). Oncotype score low (RS < 11) = 25% (114/448), intermediate (RS 11-25) = 59% (265/448) and high (RS > 25) = 15% (69/448). Grade 1 = 34% (153/448), Grade 2 = 50% (225/448) and Grade 3 = 15% (70/448). There was statistical significance in the mean difference in RS between the levels of grade and the levels of HR. The mean differences in RS between Grades 3 versus 1 was 10.9 (p < 0.001); between Grades 3 versus 2 was 8.3 (p < 0.001); and between Grades 2 versus 1 was 2.6 (p = 0.026). The mean Oncotype RS for grades 1,2,3 was 13.87 vs 16.33 vs 28.24 respectively. HR status was categorized as 51-100% (strong HR+), 11-50% (intermediate), or 1-10% (weak). Comparing strong HR+ versus intermediate/weak, the mean differences in RS were -41.4 and -35.7, respectively (p < 0.001). There were no significant differences in RS evaluated against the following parameters, patient age, tumor size, number of positive lymph nodes (0-4), LVI, or ENE. Conclusions: Tumor grade and HR status had strong association with the Oncotype RS as expected. Grade 3 tumor associated with high RS (mean 28). Intermediate HR (11-50%) associated with much higher RS = 52 similar to weak HR (1-10%) = 58. We report that RS had no correlation to patient age, tumor size or even LN status. The LN and RS association may have been impacted by low (14%) LN positive status (reportable in the modern era). Our study adds to the data that biology trumps size (traditional risk factors) while posing the question does intermediate HR status (11-50%) deserve recognition as a more substantial risk factor than previously considered.
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