A CASE OF ROSAI-DORFMAN DISEASE PRESENTING AS A PULMONARY ARTERY MASS

SESSION TITLE: Pathology Under the Microscope SESSION TYPE: Case Reports PRESENTED ON: 10/19/2022 11:15 am - 12:15 pm INTRODUCTION: Rosai-Dorfman disease (RDD) is a rare, idiopathic, nonmalignant lymphohistiocytic proliferative disorder that presents with lymphadenopathy and less commonly with extranodal involvement (1). This is a case of a patient found to have a pulmonary artery mass and bone lesions consistent with RDD. CASE PRESENTATION: A 33-year-old female with COVID pneumonia presented with one week of dyspnea, myalgias, and chills. She developed hypoxia requiring 2L of supplemental oxygen. Physical exam was benign and without lymphadenopathy. CT angiography demonstrated a well circumscribed 2.3cm x 2.1cm eccentric filling defect concerning for a pulmonary embolism versus vascular mass. She had a normal troponin and brain natriuretic peptide. Echocardiogram showed normal left ventricular ejection fraction and right ventricular size and function. Lower extremity dopplers were negative for acute deep venous thrombosis. Cardiac MRI demonstrated a mass in the posterior aspect of the proximal main pulmonary artery superior to the pulmonic valve measuring 1.9cm x 1.6cm that was consistent with a benign cardiac tumor. Patient was discharged and underwent sternotomy and excision of the mass one week later. Pathology showed histiocytosis consistent with RDD. Post-operatively she developed recurrent fevers and imaging showed bony lesions in her lumbar spine, maxilla, and skull base. Pathology from an IR guided biopsy of the lumbar lesion was suggestive of RDD. DISCUSSION: RDD is a rare, nonmalignant lymphohistiocytic proliferative disorder that usually involves lymph nodes. Concurrent nodal and extranodal involvement has been reported in 43% of cases while isolated extranodal involvement has been reported in 23% of cases. Common extranodal sites include cutaneous, soft tissue, upper respiratory tract, bone, and central nervous system (1). There are only a few cases reported of pulmonary artery involvement. These cases include a patient with RDD invading the pulmonary trunk and aorta who required surgical resection and reconstruction due to impending right ventricular failure (2) and a young woman with RDD causing nearly complete obstruction of the main pulmonary artery resulting in severe pulmonary hypertension and heart failure who required debulking (3). This case demonstrates RDD involving the main pulmonary artery and bones which was incidentally discovered when the patient was hospitalized for COVID pneumonia. RDD has a benign course but when the pulmonary artery is involved, patients often require surgical excision. CONCLUSIONS: RDD is a benign proliferation of histiocytes that most commonly presents with cervical lymphadenopathy. Extranodal involvement has been reported but pulmonary artery involvement is rare. RDD has a benign course, but pulmonary arterial involvement often requires surgical excision. Reference #1: Gaitonde, S. (2007). Multifocal, extranodal sinus histiocytosis with massive lymphadenopathy: an overview. Archives of pathology & laboratory medicine, 131(7), 1117-1121. Reference #2: Prendes, B. L., Brinkman, W. T., Sengupta, A. L., & Bavaria, J. E. (2009). Atypical presentation of extranodal Rosai-Dorfman disease. The Annals of thoracic surgery, 87(2), 616-618. Reference #3: Walters, D. M., Dunnington, G. H., Dustin, S. M., Frierson, H. F., Peeler, B. B., Kozower, B. D., … & Lau, C. L. (2010). Rosai-Dorfman disease presenting as a pulmonary artery mass. The Annals of thoracic surgery, 89(1), 300-302. DISCLOSURES: No relevant relationships by Veena Dronamraju Advisory Committee Member relationship with Nabriva Please note: 1 day Added 03/14/2022 by Rohit Gupta, value=Consulting fee No relevant relationships by MARUTI KUMARAN no disclosure on file for Bilal Lashari;No relevant relationships by Parth Rali No relevant relationships by Stephanie Tittaferrante No relevant relationships by Yoshiya Toyoda

Rox index predicts intubation in patients with COVID-19 pneumonia and moderate to severe hypoxemic respiratory failure receiving high flow nasal therapy

Introduction Use of high flow nasal therapy (HFNT) to treat COVID-19 pneumonia has been greatly debated around the world due to concern for increased healthcare worker transmission and delays in invasive mechanical Ventilation (IMV). Herein we analyze the utility of the ROX index to predict the need and timing for IMV in a retrospective analysis of patients with COVID-19 with moderate to severe hypoxemic respiratory failure treated with HFNT. Methods This was a retrospective analysis of 129 consecutive patients with COVID-19 admitted to Temple University Hospital in Philadelphia, Pennsylvania, from March 10, 2020, to May 17, 2020 with moderate to severe hypoxemic respiratory failure treated with High Flow nasal therapy (HFNT). HFNT patients were divided into two groups: HFNT only and HFNT progressed to IMV. The primary outcome was the ability of the ROX index to predict the need of IMV. Secondary outcomes were mortality, rates of intubation, length of stay (LOS) and rates of nosocomial infections in our cohort treated with HFNT were also reported. Results 837 patients with COVID-19 were screened, 129 met inclusion criteria. The mean age was 60.8(+13.6) years, BMI 32.6(+8), 58(45 %) were female, 72(55.8%) were African American, 40 (31%) Hispanic. 48 (37.2%) were smokers. Of the 129, 89 were HFNT only group whereas 40 in the HFNT progressed to IMV group. Mean time to intubation was 2.5 days(+ 3.3). The 89 HFNT only patients had a significant improvement in ROX from initiation of HFNT at all recorded time points. In contrast, the ROX in HFNT progressed to IMV patients remained unchanged or decreased over time. ROX index of less than 5 at HFNT initiation was predictive of progression to IMV (OR = 2.137, p = 0,052). Any decrease in ROX index after HFNT initiation was predictive of intubation (OR= 14.67, p <0.0001). In multivariate analysis, ΔROX (<=0 versus >0), peak D-dimer >4000 and admission GFR < 60 ml/min were very strongly predictive of need for IMV (ROC = 0.86, p=0.001). Mortality was 11.2% in HFNT only group versus 47.5% in the HFNT progressed to IMV group (p = 0.0001). Mortality and need for pulmonary vasodilators were higher in the HNFT progressed to IMV group. Conclusion ROX index is a valuable, noninvasive tool to evaluate patients with moderate to severe hypoxemic respiratory failure in COVID-19 treated with HFNT. ROX helps predicts need for IMV and thus limiting morbidity and mortality associated with IMV.

Thromboelastography (TEG) in COVID-19 patients-not all hypercoagulable

Introduction COVID-19 can lead to a severe inflammatory response and cytokine storm, which is associated with activation of blood coagulation, platelets, and endothelium leading to a severe prothrombotic state. Recent studies have interpreted TEG parameters of increased maximum amplitude (MA) and alpha angle (AA) as indicating a hypercoagulable pattern in patients with COVID-19. The definition of hypercoagulability in literature has been variable while some have used increased MA, others used increased coagulation index (CI) as a surrogate for a hypercoagulable state. Here we report our center experience using TEG to evaluate coagulation in COVID-19 patients. Methods Retrospective analysis of 37 critically ill patients that were evaluated using TEG on a single occasion along with standard coagulation tests. We defined hypercoagulable pattern as CI > 3;hypocoagulable pattern was defined as CI <-3;and normal pattern if CI was between-3-3. Results TEG patterns were interpreted as hypercoagulable in 5 (13.5%), normal in 22 (59.5%) and hypocoagulable in 10 (27%) patients using the TEG coagulation index (CI). MA and AA were elevated in 13 (35.1%) and 10 (27%) patients, respectively, and both were elevated in 8 (21.6%). Discussion Our results show a normal TEG pattern in most of our critically ill COVID-19 patients based on CI (Figure 1);only 5 (13.5%) showed a hypercoagulable pattern. These findings differ from previous reports of TEG in COVID-19 patients, where a hypercoagulable TEG pattern was shown in 83-90% of patients, in these reports interpretation of hypercoagulability was based on AA or MA. We used the CI to define a hypercoagulable state, which has been used to define hypercoagulability in orthopedic surgery and during pregnancy. An elevated MA or AA was seen in only 15 (40%) of our patients. Plasma fibrinogen, an acute-phase reactant, is also elevated in COVID-19 patients. The mean fibrinogen level in our patients was 364 mg/dl, which is lower than those reported by Panigada and Mortus, where mean fibrinogen levels were 680 and 740 mg/dl, respectively. The high MA may reflect the high fibrinogen observed in COVID-19 patients and this may explain the differences in the number of patients considered as “hypercoagulable” in our cohort compared to others. Conclusion;Our study in COVID-19 patients advances a caution in the interpretation of TEG parameters and its use as an indicator of a hypercoagulable state in COVID-19 patients.

Chest CT characteristics of patients admitted with COVID-19 pneumonia

Rationale: The novel coronavirus disease-19 (COVID-19) has presented major challenges for global health systems. Given limited availability of diagnostic testing and delays in test results during the first wave of the pandemic, our hospital used computed tomography (CT) to risk stratify patients with suspected COVID-19. The aim of this study was to describe the various patterns of disease on chest CT and relate them to chest x-ray (CXR) findings. Methods: This is a retrospective review of 559 symptomatic patients infected with COVID-19 (diagnosed by real-time reverse transcription polymerase chain reaction) admitted from March 2020 to May 2020 at Temple University Hospital (Philadelphia, PA) who received admission CXR and chest CT scans that were performed within 24 hours of admission. Scans were independently reviewed by a group of radiologists. CXRs was interpreted as “consistent with COVID-19” if there were lower lobe peripheral opacities. Chest CTs were evaluated for the presence of ground glass opacities, consolidations, interlobular septal thickening, centrilobular nodules, and crazy paving pattern. Chest CT was also assessed for background lung disease (emphysema, interstitial lung disease). Results: Of the 559 patients, median age was 58 years old, 55.5% were female, and 56.7% were African American. Median BMI was 31.61. Median duration of symptoms at time of chest imaging was 5 days. 153 (27.4%) of patient's admission CXR was not consistent with COVID-19. Of those, 124 (81%) had abnormalities on chest CT. Median number of lobes involved with disease on CT was 3.8 and 317 patients (56.7%) had all 5 lobes with disease. The most common abnormalities found were ground glass opacities (n=507, 90.7%), consolidations (n=224, 40%) and centrilobular nodules (n=127, 22.7%). Less common findings included pleural effusion (n=62, 11.8%), lymphadenopathy (n=55, 9.8%), pericardial effusion (n=24, 4.2%), and pneumothorax (n=3, 0.53%). Of note, 82 (14.7%) patients were found to have emphysema, and 2 (0.35%) were found to have interstitial lung disease. Conclusion: We present one of the largest reviews of CT scans in patients admitted for COVID-19. The majority of our population had significant burden of disease on CT at time of presentation. Ground glass opacities and consolidations were the predominant findings. Most patients did not have background emphysema or interstitial lung disease. The fact that many patients with normal CXR had abnormalities on chest CT highlights the utility of chest CT in evaluating patients with COVID-19.

Ct characteristics of COVID-19 associated with adverse outcomes

Rationale: The novel coronavirus disease-19 (COVID-19) has overwhelmed global healthcare systems. It would be beneficial to identify clinical signs that predict adverse outcomes to anticipate clinical deterioration and optimize management. COVID-19 has presented with a variety of patterns on computed tomography (CT) and these findings may assist in disease stratification. This study aims to identify potential CT characteristics that may portend adverse outcomes. Methods: This is a retrospective review of 559 symptomatic patients infected with COVID-19 admitted from March 2020 to May 2020 at Temple University Hospital (Philadelphia, PA) who received thorax CT scans on admission. These scans were independently reviewed by a chest radiologist and evaluated for the presence of ground glass opacities, consolidations, interlobular septal thickening, enlarged pulmonary artery (PA) diameter, centrilobular nodules, and crazy paving pattern. Common CT findings were then associated with a combined adverse inpatient outcome (requiring high-flow oxygen, mechanical ventilation, and/or death) through univariate and multivariate logistic regression. Results: Of the 559 patients, 182 (32.6%) required high-flow oxygen, mechanical ventilation, and/or died. The cohort with adverse outcomes were older (mean age 65.0 years vs 56.7 years, p<0.0001), but had statistically similar gender, BMI and duration of symptoms compared to the cohort without adverse outcomes. The adverse outcome cohort had more COPD (18.7% vs 8.2%) but had statistically similar proportions of hypertension, diabetes, asthma, coronary artery disease, and congestive heart failure. On multivariate logistic regression, a PA diameter greater than 30mm (OR 1.056 [95% CI 1.015-1.097], p=0.0064), segmental consolidations (OR 2.359 [95% CI 1.446-3.848], p=0.0009), and non-segmental consolidations (OR 2.441, [95% CI 1.440-4.140], p=0.0009) were found to be significant predictors of adverse inpatient outcomes of either requiring high-flow nasal cannula, mechanical ventilation, or death. Conclusion: In symptomatic COVID-19 patients, enlarged PA diameter and consolidations on chest CT were associated with worse outcomes. These findings are likely representative of advanced pulmonary involvement and may be predictors of patients who require more aggressive upfront therapy. Multicenter analysis would be beneficial to confirm these findings.