AbstractBackgroundPrimary cutaneous B‐cell lymphoma (PCBCL) is a group of B‐cell lymphomas of the skin containing primary cutaneous follicle centre lymphoma (PCFCL) and marginal zone B‐cell lymphoma/lymphoproliferative disorder (PCMZL), which are indolent, and diffuse large B‐cell lymphoma, leg type (PCDLBCL‐LT), which is aggressive.ObjectivesTo evaluate treatment outcomes between PCBCL subtypes after treatment with rituximab and to compare the efficacy of intralesional against systemic rituximab in indolent subtypes.MethodsA search for patients diagnosed with PCBCL and treated with rituximab (systemic or intralesional) across all Mayo Clinic sites was performed, yielding 39 patients.ResultsEight patients had PCFCL (six treated intralesionally and two systemically), 11 had PCMZL (four intralesional, eight systemic with one dual‐treated) and 20 had PCDLBCL‐LT (all systemic). The average age at diagnosis was 62.1 years (SD = 15.1), with average follow‐up of 1852.6 days (SD = 1473.2). 69.2% of all patients treated with any form of rituximab experienced a complete response (100% PCFCL, 81.8% PCMZL and 50% PCDLBCL‐LT). When comparing all three subtypes, a significant difference was seen in overall treatment response (p = 0.022), and progressive disease rates (p = 0.015), but not in retreatment with rituximab (p = 0.440), time to retreatment (p = 0.757), recurrence (p = 0.907) or survival (p = 0.093). In the indolent subtypes, no difference in overall treatment response (p = 1.000), progressive disease rates (p = 1.000), recurrence (p = 0.650), rituximab retreatment (p = 0.650) or time to retreatment (p = 0.724) was observed.ConclusionsThis study suggests that rituximab, as systemic therapy and intralesional therapy, is effective in the management of PCBCL, and that intralesional therapy should be considered before more aggressive therapy in indolent disease.
A full-term female was admitted at 3 days of life with a worsening rash since birth, concerning for infection. She developed clinical seizures and was transferred to our facility. She was admitted to the pediatric hospital medicine service and diagnostic workup was expanded with several specialists consulted. Presumptive diagnosis was made clinically, with definitive diagnosis established thereafter.
Pulmonary Pathology Society Survey on Practice Approaches in the Histologic Diagnosis of Fibrotic Interstitial Lung Disease: Consensus and Opportunities
Context.— The pathologic diagnosis of usual interstitial pneumonia (UIP) remains a challenging area, and application of histologic UIP guidelines has proved difficult. Objective.— To understand current practice approaches by pulmonary pathologists for the histologic diagnosis of UIP and other fibrotic interstitial lung diseases (ILDs). Design.— The Pulmonary Pathology Society (PPS) ILD Working Group developed and sent a 5-part survey on fibrotic ILD electronically to the PPS membership. Results.— One hundred sixty-one completed surveys were analyzed. Of the respondents, 89% reported using published histologic features in clinical guidelines for idiopathic pulmonary fibrosis (IPF) in their pathologic diagnosis; however, there was variability in reporting terminology, quantity and quality of histologic features, and the use of guideline categorization. Respondents were very likely to have access to pulmonary pathology colleagues (79%), pulmonologists (98%), and radiologists (94%) to discuss cases. Half of respondents reported they may alter their pathologic diagnosis based on additional clinical and radiologic history if it is pertinent. Airway-centered fibrosis, granulomas, and types of inflammatory infiltrates were considered important, but there was poor agreement on how these features are defined. Conclusions.— There is significant consensus among the PPS membership on the importance of histologic guidelines/features of UIP. There are unmet needs for (1) consensus and standardization of diagnostic terminology and incorporation of recommended histopathologic categories from the clinical IPF guidelines into pathology reports, (2) agreement on how to incorporate into the report relevant clinical and radiographic information, and (3) defining the quantity and quality of features needed to suggest alternative diagnoses.
The impact of resilience on headache disability as measured by the Migraine Disability Assessment (MIDAS)
Abstract Objective Our objectives were to examine cross‐sectional correlations of headache disability with measures of resilience, anxiety, and depression, and to determine if resilience modified the association between headache severity/frequency and disability. Background Resilience is associated with quality of life and functioning among patients with chronic conditions. We investigated whether resilience strongly mitigates headache‐related disability as measured by the Migraine Disability Assessment (MIDAS). Methods We prospectively recruited 160 patients with primary headache disorders seen in a tertiary headache medicine program between February 20, 2018 and August 2, 2019. Each participant completed the MIDAS, Conner Davidson Resilience Scale (CDRS‐25), Patient Health Questionnaire‐9 (PHQ‐9), Generalized Anxiety Disorder‐7 (GAD‐7), and WHO‐5 Well‐Being Index. Results The CDRS‐25 score was negatively correlated with the total MIDAS ( r = −0.21, p = 0.009), GAD‐7 ( r = −0.56, p < 0.001), and PHQ‐9 scores ( r = −0.34, p < 0.001). Well‐being inversely correlated with disability ( r = −0.37, p < 0.001). Increases in anxiety and depression increased the odds of disability. A 1 point increase in the CDRS‐25 score decreased the odds of being severely disabled by 4% (OR = 0.96, 95% CI: 0.94 to 0.99, p = 0.001). However, the CDRS‐25 score did not significantly moderate the association between headache days and disability. Conclusion Traits associated with resilience decreased the odds of severe disability from headaches, whereas anxiety, depression, and headache frequency were strongly associated with higher disability from headache.
Abstract Number ‐ 278: Transient Ischemic Attack Evaluation Pathways: Inpatient Versus Expedited Outpatient Comparative Analysis
Introduction Transient Ischemic Attack (TIA) evaluation requires urgent etiology identification and targeted stroke prevention, traditionally performed via hospital admission. The literature suggests that outpatient evaluation may be safe and cost‐effective. Hence, we implemented an expedited outpatient pathway (OP) for patients presenting to our institution’s Emergency Department (ED) with TIA. To further guide future practice, we compared clinical features and outcomes between expedited OP and our well‐established inpatient pathway (IP). Methods We implemented an algorithm of direct communication between ED providers and Vascular Neurologists for all TIA patients. Certain screening studies (head CT, CT angiogram, basic laboratories) and acute therapies were implemented in the ED, whereas others (brain MRI, echocardiogram, etc) and Vascular Neurology consultations were expedited outpatient within 48–72 hours.We included all patients that underwent OP evaluation from May 2021‐May 2022. Further, we extracted patients with a final diagnosis of TIA and compared them with IP TIA patients hospitalized (2017‐2021), matched by age, gender, and clinical severity (ABCD2). Descriptive statistics were used to summarize patient demographics, clinical data, treatments, and 3‐month outcomes. Continuous variables were summarized with mean and standard deviations while categorical variables were shown as counts and percentages. Chi‐square test was used for categorical variables and Mann‐Whitney U test was used to compare continuous variables. P‐values < 0.05 were considered statistically significant and all tests were two‐sided. Statistical Analysis was completed in R 4.0.3. Results Of 43 patients who underwent expedited OP TIA workup, TIA or minor stroke was diagnosed in 21/43 (48.9%). 18 TIAs underwent comparative analyses. Mean symptom duration was longer in the IP (2.06 hours) than OP (0.99 hours), p = 0.370%. TIA was more likely to be diagnosed in the OP 14/18 (78%) versus IP 12/18 (67%), (p = 0.423). OP had more transesophageal echocardiograms (17% vs 5%, p = 0.630) and longer duration of ambulatory cardiac monitoring (mean 574.3 vs 381.7 hours, p = 0.100). IP patients were more likely to get started on anticoagulation compared to OP (38% vs 11%, p = 0.258) and high‐intensity statin (38% vs 17%, p = 0.260). All laboratories, echocardiograms and brain MR imaging were performed significantly sooner for the IP (p< 0.05 for all). IP had higher rate of return ED visits (22%) and readmissions at 3 months (11%), whereas OP had none. OP had no deaths or recurrent strokes, whereas 11% and 5.6% respectively were noted in the IP at 3 months (p = 0.310, p = 0.310). Conclusions Our study shows that routine IP evaluation for patients presenting with TIA in the ED may not offer diagnostic superiority nor lead to improvement of short‐term clinical outcomes, as compared to the expedited OP evaluation. Careful evaluation of TIA patients in the ED followed by expedited OP evaluation might be a plausible approach to help lower costs and avoid hospitalization without compromising the safety of these patients.
Clinical Outcomes of Stereotactic Radiosurgery-Related Radiation Necrosis in Patients with Intracranial Metastasis from Melanoma
Background: Radiation necrosis (RN) is a clinically relevant complication of stereotactic radiosurgery (SRS) for intracranial metastasis (ICM) treatments. Radiation necrosis development is variable following SRS. It remains unclear if risk factors for and clinical outcomes following RN may be different for melanoma patients. We reviewed patients with ICM from metastatic melanoma to understand the potential impact of RN in this patient population. Methods: Patients who received SRS for ICM from melanoma at Mayo Clinic Arizona between 2013 and 2018 were retrospectively reviewed. Data collected included demographics, tumor characteristics, radiation parameters, prior surgical and systemic treatments, and patient outcomes. Radiation necrosis was diagnosed by clinical evaluation including brain magnetic resonance imaging (MRI) and, in some cases, tissue evaluation. Results: Radiation necrosis was diagnosed in 7 (27%) of 26 patients at 1.6 to 38 months following initial SRS. Almost 92% of all patients received systemic therapy and 35% had surgical resection prior to SRS. Patients with RN trended toward having larger ICM and a prior history of surgical resection, although statistical significance was not reached. Among patients with resection, those who developed RN had a longer period between surgery and SRS start (mean 44 vs 33 days). Clinical improvement following treatment for RN was noted in 2 (29%) patients. Conclusions: Radiation necrosis is relatively common following SRS for treatment of ICM from metastatic melanoma and clinical outcomes are poor. Further studies aimed at mitigating RN development and identifying novel approaches for treatment are warranted.
Clinical and histopathological features of hypereosinophilic syndrome with cutaneous involvement: The Mayo Clinic Experience
AbstractBackgroundHypereosinophilic syndrome (HES) encompasses a group of diseases with blood hypereosinophilia and eosinophil‐mediated organ dysfunction. HES‐associated skin abnormalities, termed cutaneous HES (cHES) here, may influence diagnosis of HES. We sought to better define clinical and histopathological features of cHES.MethodsWe retrospectively reviewed clinical records and cutaneous histopathology of adult patients with HES evaluated at our institution from 2007 to 2018.ResultsForty‐one percent (61/150) patients with HES had cHES. The most common clinical morphologies were urticarial (30%) and eczematous (26%). Skin specimens most often showed a spongiotic pattern (31%) with abundant inflammation (50%) including eosinophils (85%). Two specimens (8%) showed interstitial granulomatous dermatitis, and two specimens showed eosinophilic fasciitis (8%). Vasculitis was not identified in any specimen. Eighty‐four percent of patients with cHES had ≥1 other organ system involved: pulmonary 41%, ENT 26%, and nervous 23%. Sixty percent (53/89) of non‐cHES patients had at least two organ systems involved. Cardiac or gastrointestinal involvement was more common in non‐cHES than cHES (p < 0.05).ConclusionOur review confirms that there are no specific clinical or histopathological cHES patterns, but HES should be considered in patients who have eczematous or urticarial reactions of unknown etiology and persistent peripheral hypereosinophilia.
Abstract WP37: Prehospital Stroke Scales Outperform National Institutes Of Health Stroke Scale In Predicting Large Vessel Occlusion In A Large Telestroke Network
Introduction: Prehospital telestroke is increasingly utilized in mobile stroke units and telemedicine-enabled ambulances for acute ischemic stroke (AIS). While the NIHSS remains the standard for telestroke assessment, simpler scales have been validated to help paramedics recognize large vessel occlusion (LVO) strokes but have not been evaluated in a traditional telestroke network. We aimed to determine the accuracy of common LVO scales in a large academic telestroke practice. Methods: This retrospective study included all telestroke consults performed in a large academic telestroke network from 2019 to 2020. Patients were excluded if no NIHSS or vessel imaging was performed. We recorded presenting NIHSS, site of vessel occlusion, and discharge diagnosis. LVO was defined as an acute occlusion of the internal carotid artery and/or M1 middle cerebral artery. The NIHSS was used to calculate 7 LVO scales (RACE, C-STAT, FAST-ED, 3I-SS, PASS, VAN, and G-FAST). Diagnostic performance was assessed by calculating sensitivity, specificity, negative predictive value (NPV), positive predictive value (PPV), and accuracy using the established thresholds of each scale. These results were compared to NIHSS at thresholds of 6, 8, and 10. Area under curve (AUC) was calculated using c-statistics by treating scales as continuous variables. Results: A total of 625 patients were included, 373/625 (59.7%) of which were diagnosed with AIS at discharge. LVO was identified in 78/625 (12.5%) patients. VAN was the most sensitive prehospital stroke scale (83.3%), whereas 3I-SS ≥ 4 was the most specific (95.2%). Both the RACE and FAST-ED scales demonstrated superior accuracy and AUC compared to the NIHSS (Table 1). Conclusions: To our knowledge, this is the first study assessing LVO scale accuracy in a large telestroke network. As use of prehospital telestroke grows, this study highlights several scales that may allow for faster prehospital triage decisions without sacrificing accuracy.
Abstract TP45: Demonstration Of High Diagnostic Accuracy Of Cerebral Ischemia In A Large Academic Hub And Spoke Telestroke Network
Introduction: Telestroke facilitates emergent assessment of patients with suspected acute ischemic stroke (AIS) to identify candidates for acute interventions. The diagnostic accuracy of the telestroke consultation of a large academic hub and spoke telemedicine network has yet to be investigated. The goal of our study is to determine the diagnostic accuracy of the initial telestroke consultation. Methods: This is an IRB-exempt retrospective study including all patients evaluated for cerebral ischemia via video telestroke consultation in a large academic hub and spoke telemedicine network from 2019 to 2020. Detailed chart review was conducted to identify initial suspected diagnosis and final diagnosis. Cerebral ischemia was defined as AIS and transient ischemic attack (TIA). All other diagnoses were defined as stroke mimics. Data was organized into continuous and categorical variables. Sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), accuracy, area under the curve (AUC) and likelihood ratio (LR+) for the initial telestroke consultation diagnosis were calculated while using the final diagnosis as the gold standard diagnosis. Results: A total of 1043 patients met inclusion criteria. Final diagnosis of cerebral ischemia was made in 63.5% of all patients (539/1043 with AIS,123/1043 with TIA). Stroke mimic was diagnosed in 36.5% patients (381/1043). The sensitivity and specificity of telestroke evaluation for diagnosis of cerebral ischemia were 97.1% and 81.4%, respectively. PPV was 90.1%, and NPV was 94.2%. Overall diagnostic accuracy was 91.4% with a LR+ of 5.21 and AUC of 0.89. The most common stroke mimics were metabolic encephalopathy (13.7%, 52/381), migraine (10.8%, 41/381) and seizure (10.5%, 40/381). Conclusions: This study highlights the high diagnostic accuracy of telestroke providers to diagnose cerebral ischemia and stroke mimic in a large academic hub and spoke network. Further research is needed to investigate methods to improve our diagnostic accuracy further, as well as explore the application of tele-neurology in the non-stroke setting.
GFR estimated with creatinine rather than cystatin C is more reflective of the true risk of adverse outcomes with low GFR in kidney transplant recipients
ABSTRACT Background Serum cystatin C–based estimated glomerular filtration rate (eGFRcys) generally associates with clinical outcomes better than serum creatinine–based eGFR (eGFRcr) despite similar precision in estimating measured GFR (mGFR). We sought to determine whether the risk of adverse outcomes with eGFRcr or eGFRcys was via GFR alone or also via non-GFR determinants among kidney transplant recipients. Methods Consecutive adult kidney transplant recipients underwent a standardized GFR assessment during a routine follow-up clinic visit between 2011 and 2013. Patients were followed for graft failure or the composite outcome of cardiovascular (CV) events or mortality through 2020. The risk of these events by baseline mGFR, eGFRcr and eGFRcys was assessed unadjusted, adjusted for mGFR and adjusted for CV risk factors. Results There were 1135 recipients with a mean baseline mGFR of 55.6, eGFRcr of 54.8 and eGFRcys of 46.8 ml/min/1.73 m2 and a median follow-up of 6 years. Each 10 ml/min/1.73 m2 decrease in mGFR, eGFRcr or eGFRcys associated with graft failure [hazard ratio (HR) 1.79, 1.68 and 2.07, respectively; P < .001 for all) and CV events or mortality outcome (HR 1.28, 1.19 and 1.43, respectively; P < .001 for all). After adjusting for mGFR, eGFRcys associated with graft failure (HR 1.57, P < .001) and CV events or mortality (HR 1.49, P < .001), but eGFRcr did not associate with either. After further adjusting for CV risk factors, risk of these outcomes with lower eGFRcys was attenuated. Conclusion eGFRcr better represents the true relationship between GFR and outcomes after kidney transplantation because it has less non-GFR residual association. Cystatin C is better interpreted as a nonspecific prognostic biomarker than is eGFR in the kidney transplant setting.