Changes in Kawasaki disease incidence and phenotype during the COVID-19 pandemic

INTRODUCTION. The aetiology of Kawasaki disease (KD) remains unknown. Changes in infectious exposure during the COVID-19 pandemic owing to infection prevention measures may have affected the incidence of KD, supporting the pathogenic role of an infectious trigger. The purpose of this study was to evaluate the incidence, phenotype and outcome of KD before and during the COVID-19 pandemic in Denmark. METHODS. This was a retrospective cohort study based on patients diagnosed with KD at a Danish paediatric tertiary referral centre from 1 January 2008 to 1 September 2021. RESULTS. A total of 74 patients met the KD criteria of whom ten were observed during the COVID-19 pandemic in Denmark. Alof these patients were negative for SARS-CoV-2 DNA and antibodies. A high KD incidence was observed during the first six months of the pandemic, but no patients were diagnosed during the following 12 months. Clinical KD criteria were equally met in both groups. The fraction of intravenous immunoglobulin (IVIG) non-responders was higher in the pandemic group (60%) than in the in the pre-pandemic group (28.3%), although the rate of timely administered IVIG treatment was the same in both groups (>= 80%). Coronary artery dilation was observed in 21.9% in the pre-pandemic group compared with 0% in KD patients diagnosed during the pandemic. CONCLUSION. Changes in KD incidence and phenotype were seen during the COVID-19 pandemic. Patients diagnosed with KD during the pandemic had complete KD, higher liver transaminases and significant IVIG resistance but no coronary artery involvement.Copyright © 2023, Almindelige Danske Laegeforening. All rights reserved.

Clinical Features, Laboratory Findings Outcomes in Multisystem Inflammatory Syndrome (Mis-C) Following Covid-19 Infection - a Multicenter, Observational, Ambidirectional Cohort Study in Rio De Janeiro State, Brazil

Objectives: As of March 5th, 2022, around 1.585 cases of MIS-C and 98 deaths (6,4%) were reported in Brazil. The state of Rio de Janeiro State (RJ) having 94 cases (5,9%) and 4 deaths (4,2%)1.Our aim was to evaluate clinical and laboratory features, and management of MIS-C in seven pediatric hospitals in RJ, Brazil. Method(s): Multicenter, observational, ambidirectional cohort study in seven tertiary hospitals in RJ(Brazil), assessing medical charts of pediatric inpatients (0-18 years) diagnosed with MIS-C according to WHO/CDC criteria, from August, 2020 to February, 2022. Descriptive statistics were used to analyze distributions of continuous variables, frequencies, and proportions. Result(s): A total of 112 cases of MIS-C were enrolled. The mean age was 4.2 years and thre was male predominance (59,8%). All cases had a SARS-CoV-2 contact (29.5% close contact;31.3%:positive PCR;serology:43.8%).Only 12.5% had comorbidities. Length of stay (LOS) was 7 days.Median duration of fever was 8 days. Most common symptoms were: rash(67%);gastrointestinal (67%);conjunctivitis (42%);neurological(39.6%);cardiovascular(37.5%);cervical lymphadenopathy (36.6%), and shock/hypotension(28.6%).Co-infection occurred in 3 patients. Forty-four patients fulfilled criteria for Kawasaki disease. Most patients were admitted to PICU(12;62,5%) for amedian of 2 days. Respiratory distress was seen in 18,7%;hypotension:28,6%, and shock in 23,2%. Main laboratory findings were: high C-reactive protein in 95%;D-dimer:77%, anemia:77%, thrombocytosis:63%;transaminitis:43.8%, lymphopenia:38%;hypoalbuminemia:34%;thrombocytopenia: 29%;hypertriglyceridemia:28%, and high pro-BNP in 27%. Echocardiogram was performed in 91/112 patients;abnormal in 70,3%;exhibiting myocardial dysfunction( 25%);pericardial effusion(21%);coronary dilation/aneurysms(11%) and, valvulitis (14.5%). IVIG+corticosteroids (CTC) were administered in 59.8%(67/ 112);18.6%(18/112) IVIG only;10.7%(12/112) CTC only;3.4%(4/112)biologics, and 15(13.3%) received no treatment. ASA low dose in 77.7% (87/112) and moderate/high dose in 34.8%. Oxygen support was needed in 27,7%;vasoactive amines:18,7%;dialysis:5,3%, and transfusion:18,7%.One patient died from a cytokine storm syndrome. Conclusion(s): Our study reports a higher number of MIS-C cases in RJ than the number reported to Brazilian authorities, highlighting underreporting. Our patients were younger, had fewer comorbidities, cardiovascular/gastrointestinal/renal involvement, shortest LOS in ICU, and a higher frequency of myopericarditis.

Kikuchi-Fujimoto Disease: A Case of SARS-CoV-2 Infection Triggering the Rare Disease.

Kikuchi-Fujimoto disease (KFD), or histiocytic necrotizing lymphadenitis, is a rare, benign, and self-limited disease caused by subacute necrotizing regional lymphadenopathy. The etiology is unknown, although virus and autoimmune mechanisms have been proposed. Patients develop enlarged lymph nodes, fever, and, less frequently, extranodal signs. No specific laboratory test contributes to the diagnosis, and lymph node biopsy is the gold standard to define the diagnosis. The treatment is generally with supportive therapy and usually is spontaneously resolved within six months.  In this article, the authors present the case of a 41-year-old female with mild SARS-CoV-2 (severe acute respiratory syndrome coronavirus 2) infection 10 weeks before she was admitted to the emergency department (ED) due to cervical lymphadenopathies and fever lasting over three weeks. Physical examination revealed multiple lymphadenopathies on the submandibular and jugular regions, cutaneous rash, and hepatosplenomegaly. Blood tests showed elevated acute phase proteins, thrombocytopenia, and increased transaminases and lactate dehydrogenase (LDH). Computed tomography (CT) of the neck showed multiple adenopathies at levels I, II, III, and IV according to the Classification for Lymph Nodes from the American Head and Neck Society and American Academy of Otolaryngology on the right side. Excision biopsy was performed and histopathological examination confirmed KFD. A comprehensive analysis performed was unrevealing of an infectious or autoimmune cause and was assumed to be most likely triggered by SARS-CoV-2 infection given the timeframe correlation. KFD diagnosis is challenging and there are few reported cases of association with SARS-CoV-2 in the literature. Although further investigation is still needed to better understand the relation between them, it is important that physicians take SARS-CoV-2 infection and vaccination into consideration in KFD's differential diagnosis.

Hydroxychloroquine Induced DRESS (Drug Rash with Eosinophilia and Systemic Symptoms Syndrome) Mimicking SARS-COV-2 Illness: A Case Report with Review

Background: In the current pandemic of COVID-19, hydroxychloroquine (HCQ) is recom-mended as an experimental drug for prophylaxis and treatment of the illness. Although it is a safe drug, it can rarely produce a severe drug reaction 'drug rash with eosinophilia and systemic symptoms syndrome (DRESS)', and to differentiate it from systemic viral infections is challenging. Case Presentation: A 45-year old male nurse working in a COVID-19 ward consumed HCQ weekly for two weeks for prevention of SARS-COV-2 illness. He presented with fever, pruritic maculopapular palmar rash, cervical lymphadenopathy for 12 hours and was quarantined as a suspected COVID-19 case. His laboratory tests revealed lymphopenia, eosinophilia, atypical lymphocytes, raised liver en-zymes along with IgM negative, IgG positive rapid antibody test of SARS-COV-2. However, his throat swabs for SARS-COV-2 by real-time PCR were negative on day 1 and 7. He was finally diagnosed as definite DRESS based on the RegiSCAR score of six. He responded to levocetirizine 5 mg OD and oral prednisolone 60 mg daily tapered over 7 days. Conclusion(s): DRESS due to HCQ is 'probable', 'of moderate severity', and 'not preventable' adverse effect mimicking SARS-COV-2 illness.Copyright © 2021 Bentham Science Publishers.

Optimal approach to T-cell ALL

T-lineage acute lymphoblastic leukemia (T-ALL) is curable for most children and adolescent and young adult patients with contemporary frontline chemotherapy regimens. During the past decade, improved survival rates have resulted from the optimization of frontline chemotherapy regimens, the use of minimal residual disease (MRD) assessment for evaluating a patient's risk for relapse, and the intensification of treatment based on the persistence of MRD. Optimization of initial therapy is critical because relapsed T-ALL after initial intensive chemotherapy is incurable for most adult patients. Current T-ALL salvage chemotherapy regimens are minimally effective, and unlike in B-cell ALL, there are no approved antibody therapies or chimeric antigen receptor T-cell therapies for relapsed disease. Immunotherapy and small-molecule inhibitors are beginning to be tested in relapsed T-ALL and have the potential to advance the treatment. Until effective salvage strategies are discovered, however, intensive frontline therapy is required for cure. In this article I review the current frontline chemotherapy regimens for adult patients with T-ALL, summarize the novel targeted and immune therapeutics currently in early-phase clinical trials, and outline how these therapies are helping to define an optimal approach for T-ALL.Copyright © 2022 by The American Society of Hematology.

Kikuchi's Disease Diagnosed by an Excisional Biopsy in a Patient With COVID-19.

COVID-19 is an ongoing pandemic caused by the novel coronavirus SARS-CoV-2. The clinical features of COVID-19 are myriad. Though it is a multisystem illness, it predominantly involves the respiratory system. There have been case reports on rare manifestations of COVID-19, of which COVID-19-related Kikuchi's disease is one of them. To our knowledge, this is the third reported case in the world. We report a lady in her late 60s with COVID-19 infection and secondary bacterial pneumonia, which necessitated ICU admission, having ongoing fever spikes with high inflammatory markers and leukopenia. She was also found to have tender cervical lymphadenopathy on the third week of illness, whose biopsy revealed histiocytic necrotizing lymphadenitis in keeping with Kikuchi's disease. The patient had an uneventful recovery in two weeks without any intervention. The pathophysiology of COVID-19-related Kikuchi's disease is unclear. However, COVID-19 is a viral illness that involves changes in interleukins. The latter is postulated in Kikuchi's disease.

It's not the shunt: an atypical cause of fever in an infant with hydrocephalus

Case Report:We present a 5-year-old male with two days of fever, cough, vomiting, and loose stools. His history is significant for premature birth (35 weeks gestational age) and shunted hydrocephalus. A ventriculoperitoneal (VP) shunt was placed 6 days prior to presentation. Parental report included episodes of post-tussive, nonbloody, non-bilious emesis, poor oral intake, tachypnea, and increased work of breathing. Physical examination demonstrated a dehydrated infant with sunken fontanelles. He had no notable rash, no lymphadenopathy, and clear conjunctiva. His VP shunt site appeared normal without swelling or erythema. Initial evaluation showed elevated inflammatory markers -ESR 51 and CRP 12.32 mg/dL. A viral respiratory PCR panel returned positive for coronavirus (not SARS-CoV-2). A head CT scan and shunt radiography series showed no abnormalities with his shunt. The following morning, Radiology reported an incidental retropharyngeal fluid collection on a re-read of the patient's initial CT scan. A neck CT was obtained and demonstrated a fluid pocket with secondary mass effect in addition to bilateral cervical lymphadenopathy. Screening blood cultures were negative. The patient remained febrile (tmax 103.6F) and developed a transaminitis (ALT 264.9, AST 654), elevated fibrinogen 476, elevated INR 1.4, and low albumin 2.1. Abdominal ultrasound showed a normal the liver and biliary tract. His transaminitis resolved without treatment. The next day, the patient developed lip erythema and conjunctival injection. An echocardiogram showed a dilated right coronary artery (z-score of 3.59) and his inflammatory markers (ESR 26, CRP 9.63) remained elevated. Treatment was initiated with IVIG and moderate-dose aspirin. The patient defervesced, and he remained afebrile for over 48 hours prior to discharge. A repeat echocardiogram 2 days later showed a slight reduction in coronary artery dilatation (z-score 3.39). Hewas discharged on lowdose aspirin, and followed up with cardiology as an outpatient. Kawasaki's Disease (KD) is most common in children from ages 1 to 4 years and is classically characterized by persistent fever with a constellation of symptoms including limbal sparing conjunctivitis, cervical lymphadenopathy, polymorphous rash, strawberry tongue, oral changes, and extremity changes. Our patient presented at a younger age with a concurrent diagnosis of coronavirus upper respiratory tract infection. His atypical hospital course and incidental finding of retropharyngeal edema and transaminitis increased the clinical suspicion for KD. His symptoms rapidly improved after administration of IVIG. Younger patients are at an increased risk for severe complications of KD including coronary aneurysm. KD has been shown in the literature to have an association with coronavirus infection as well as presentation with retropharyngeal edema. Clinicians should consider KD in their differential even if patients do not meet all criteria for diagnosis on initial presentation. Copyright © 2023 Southern Society for Clinical Investigation.

Kawasaki-like multisystem inflammatory syndrome in an adult filipino with covid-19 infection: a case report

Objectives: Several case series have been identified describing adult patients with a secondary Multisystem Inflammatory Syndrome (MIS) after a recent COVID-19 infection, presenting with features similar to Kawasaki Disease (KD). This paper aims to describe the first case of Kawasaki-like presentation in an adult Filipino with COVID-19 infection. Method(s): A case of Kawasaki-like presentation in an adult Filipino with COVID-19 infection was reviewed in a tertiary hospital. Result(s): This is a case of a 38-year- old Filipino woman who presented with fever, malaise, dry cough, sore throat, diarrhea and rashes. On examination, she had classic features of KD: non-exudative conjunctivitis, cracked lips, edema of the hands and feet, palmar erythema, diffuse rash, and cervical lymphadenopathy. Laboratory results showed anemia, leukocytosis with neutrophilia, hyponatremia, hypokalemia, transaminitis, hypoalbuminemia, and elevated inflammatory markers. COVID-19 PCR test was negative but serologic studies showed positivity for IgM and IgG, suggesting a recent infection. Treatment included methylprednisolone pulse therapy, intravenous immunoglobulin (IVIG) and anticoagulation, which resulted in rapid clinical improvement. Conclusion(s): As the knowledge of COVID-19 and its associated clinical features continually evolves, it is imperative to be aware of the possibility of KD as a delayed post-viral multisystem inflammatory response in adults that may warrant prompt treatment.

An Unusual case of epstein-barr virus hepatitis mistaken for multisystem inflammatory syndrome in children

Case Report: As COVID-19 infections became more common, children began presenting with multisystem inflammatory syndrome (MIS-C). It can be difficult to distinguish rare presentations of common diseases from MIS-C, especially when there has been a close contact with COVID-19. Epstein-Barr virus (EBV) is a universally common infection with 90% of individuals showing serological signs of past infection. Both MIS-C and EBV can present with similar signs and symptoms. Our case aims to remind the reader to keep in mind uncommon presentations of common viral infections which may mimic MIS-C. Case Presentation: A previously healthy 5-year-old girl presented with persistent fevers for 12 days, associated with stomatitis, vomiting, and diarrhea. Physical exam was significant for a moderately ill-appearance, small (<1 cm) left posterior cervical lymphadenopathy, and soft palate and buccal oral ulcers. Initial labs (see Table) revealed leukocytosis with reactive lymphocytes and cholestatic hepatitis with mild coagulopathy. Although she had no respiratory symptoms, CT chest revealed left upper lobe pneumonia. Abdominal ultrasound showed diffuse hepatosplenomegaly, gallbladder wall thickening, and enlarged epigastric lymph nodes. Echocardiogram showed normal systolic function and coronary arteries without dilation. Extensive viral and bacterial nasal swab and serologic testing, including for SARS-CoV-2 antibodies, was negative. On Day 2, her Monospot was positive, along with EBV viral capsid antigen IgM and IgG with the absence of EBV nuclear antigen IgG. In addition, serum PCR was positive for EBV. Management and Outcome: Due to persistent fevers on Day 3 of broad-spectrum antibiotics, coupled with a close contact with active COVID infection, she was treated with the MIS-C protocol of intravenous immunoglobulin G (IVIG), prednisone, and aspirin. Within a day of IVIG, she improved clinically and fever resolved. By discharge on Day 8, her lab values had begun to normalize. Discussion(s): EBV is known to present in children with typical infective mononucleosis symptoms such as fever, sore throat, and lymphadenopathy. However, these can be lacking which makes the diagnosis challenging. Although hepatitis is a common sequalae of EBV, EBV induced pneumonitis and stomatitis are rare, especially in immunocompetent individuals. While our patient improved after treatment with IVIG, suggesting MIS-C, we still attribute her illness to EBV, as IVIG has been shown to provide antiviral and anti-inflammatory benefit in EBV infections. This case highlights the challenge of recognizing and overtreating rare presentations of common viral infections in the face of an emerging disease such as MIS-C. Significant Laboratory Values [Table presented] Copyright © 2023 Southern Society for Clinical Investigation.

Using HScore for the evaluation of the signs of hemophagocytosis in multisystem inflammatory syndrome associated with covid-19 in children

Background/Purpose: Multisystem inflammatory syndrome associated with COVID-19 in children (MIS-C) is a rare but severe disease associated with coronavirus infection, in which various systems and organs are affected, including the heart, lungs, kidneys, brain, skin, eyes and gastrointestinal tract. One of the most severe features of this disease can be hemophagocytosis. The aim of this study is to assess the features of hemophagocytosis in MIS-C. Method(s): The retrospective study included 166 children (99 male, 67 female), aged from 4 months to 17 years (median 8.2 years), who met the WHO criteria for MIS-C. The analysis of the obtained data was performed using the STATISTICA software package, version 10.0 (StatSoft Inc., USA). Result(s): To study the signs of hemophagocytosis in patients with MIS-C they were divided into 2 equal groups: with HScore<=91 (n = 79) and with a HScore value >91 (n = 79). This division was done, since this value was associated with the severe life-threatening course of MIS-C and need in ICU admission (70.9% vs. 32.3%, P = 0.000002). Patients with HScore > 91 were more likely to have symptoms such as cervical lymphadenopathy (80.6% vs 54.1%, P = 0.0007), red dry cracked lips (63% vs 34.3%, P = 0.0007), face swelling (66.7% vs 34.7%, P = 0.001), hepatomegaly (84.2% vs 43.1%, P = 0.000000), splenomegaly (54.7% vs 43.1%, P = 0.0003), hypotension/shock (63.3% vs 25.3%, P = 0.000002), had higher levels of ESR (47 mm/h vs 34 mm/h, P = 0.0001), CRP (175.5 mg/L vs 125.8 mg/L, P = 0.01), D-dimer (2135 ng/mL vs. 1079 ng/mL, P = 0.0003), but lower levels of fibrinogen (3.1 g/L vs 5.6 g/L, P = 0.000002) erythrocytes (3.6 x 1012/L vs 4.0 x 1012/L, P = 0.000005), hemoglobin (98 g/L vs 112 g/L, P = 0.000000), and a tendency to thrombocytopenia (110 x 109/l vs 192 x 109/L, P = 0.0002) in 63.3% of patients. According to EchoCG data, signs of myocardial (45.5% vs 15.6%, P = 0.00006) and pericardial (45.5% vs 14.3%, P = 0.00002) lesions were more common in patients with HScore > 91. Patients with HScore > 91 more often needed treatment with IVIG (66.2% vs 24%, P = 0.000000), acetylsalicylic acid (65.7% vs. 47.1%, P = 0.027) and biological drugs (9.1% vs. 1.6%, P = 0.061). The average duration of hospitalization was also much longer in patients with HScore > 91 (23 days vs 14 days, P = 0.000000). Also, the identification of clinical and laboratory signs that were more common in the group of patients with HScore > 91 was performed using sensitivity and specificity analysis, and calculation of odds ratio. Results are presented in Table 1. Conclusion(s): Hemophagocytic syndrome is one of the most severe manifestations of MIS-C occuring in 35.4% of patients. It was found that HScore > 91 is associated with such a severe signs of MIS-C as myocarditis, pericarditis, hypotension/shock, and ICU admission. HScore is a simple tool that can also be used to assess the severity of MIS-C and dynamic monitoring.

Not all shortness of breath and throat pain is COVID

Case Report: A previously, healthy 18-year-old female presents to a Pediatric Emergency Medicine Department with shortness of breath, fever, and worsening throat and abdominal pain for 3 days. She had a sick contact, a teacher that tested positive for COVID-19 2 weeks prior to presentation. She denies runny/stuffy nose, cough, loss of taste/smell, or rashes/lesions. She denies any significant past medical history including allergies, as well as any history of smoking or any illicit drug use. Upon arrival to the ED, the patient was noted to be tachycardic, hypotensive and febrile. There were no desaturations. Initial physical examination revealed a generally uncomfortable female that was alert and oriented, with noted tenderness over the right anterior neck region, diffuse cervical lymphadenopathy, and painful neck range of motion. Her pharynx was noted to be erythematous without exudates or any unilateral tonsillar swelling. In the ED patient received IV fluid resuscitation and was started on norepinephrine drip, broad spectrum antibiotics. Initial lab workup revealed an anion gap metabolic acidosis, likely secondary to uremia or lactic acidosis from poor perfusion in setting of sepsis and hypovolemia. BUN and creatinine were elevated, likely due to an acute kidney injury (AKI) secondary to hypovolemia. The patient was also found to have an elevated LDH, fibrinogen, and mild elevation of AST. D-Dimer was elevated at 29 000. Covid PCR, Rapid Strep, and respiratory PCR panel were negative. Her chest X-ray (CXR) was negative and ECG showed sinus tachycardia. Given the patient's history of throat and neck pain with shortness of breath, in the setting of a septic picture, a CT scan of neck, chest, abdomen was ordered prior to transferring the patient to the PICU. CT scan of the chest revealed small patches of consolidation with ground glass opacities in the right lung apex, as well as an nearly occlusive, acute thrombosis of the anterior right facial vein. The patient's initial blood cultures grew gram negative bacilli which later were revealed to be Fusobacterium necrophorum. These findings are consistent with Lemierre's syndrome. The patient was treated in the PICU on vasopressors, heparin anticoagulation, and antibiotics for 6 days and discharged with a course of Augmentin. Lemierre's syndrome is an infectious thrombophlebitis of the internal jugular vein. First described by Andre Lemierre in 1936, it begins as a bacterial pharyngitis, generally developing into a peritonsillar abscess or other deep space neck infection with progressive erosion into the internal jugular vein. Diagnostic criteria for Lemierre's syndrome includes radiographically evidence of thrombophlebitis of the internal vein and positive blood cultures. CT and MRI can help make the diagnosis, but are not always required. Treatment is prompt intravenous antibiotics with beta-lactamase penicillins, metronidazole, clindamycin, and third generation cephalosporins. [Figure presented] Copyright © 2023 Southern Society for Clinical Investigation.

Head and neck imaging manifestations in COVID-19: collective experience of 17 months during 2nd wave of the pandemic

Background: COVID-19 is well known to result in pulmonary and multiple extra-pulmonary manifestations. Among them, head and neck manifestations were commonly recognized in the 2nd wave of the pandemic. With the growing global COVID-19 burden, imaging is of utmost importance in diagnosing the disease and its related complications. The study aims to enumerate the various head and neck manifestations and their complications in COVID-19. Additionally, in sinusitis patients, the invasion was correlated with the neutrophil-lymphocyte ratio (NLR). Result(s): A cross-sectional observational study in which total of 78 COVID-19 cases that underwent head and neck imaging were retrospectively evaluated. The cohort included 52 males (66.7%) and 26 females (33.3%) with a mean age of 46.19 years (median = 49.0, SD = 16.47). The various head and neck manifestations included non invasive rhinosinusitis (n = 48), invasive sinusitis and its complications (n = 25), nasal septal abscess (n = 1), dacryoadenitis (n = 1), pre-septal and post-septal orbital cellulitis and its complications (n = 13), otitis media, mastoiditis and its complications (n = 6), parotitis (n = 2), neck vessel thrombosis (n = 2) and cervical lymphadenopathy (n = 3). An increase in the invasive nature of sinusitis was demonstrated among patients with comorbidities and elevated NLR. Conclusion(s): Early diagnosis and management of head and neck manifestations of COVID-19 are aided by prompt imaging. It is imperative that we are armed with the knowledge of various head and neck manifestations and how they may bear semblance to other pathologies for us to ensure COVID as a differential, especially in the background of known infection. Copyright © 2023, The Author(s).

Kikuchi-Fujimoto disease following COVID-19 infection in a 13-year- old girl

Background/Purpose: Kikuchi-Fujimoto disease (KFD) is a rare, self-limited histiocytic necrotizing lymphadenitis. Although it is of uncertain aetiology, it is associated with viral infections and autoimmune diseases. Hence, it is crucial to identify KFD from other conditions with lymphadenopathy. Here we present a case of KFD after COVID-19 infection. Method(s): Medical records were traced and reviewed Results: A previously healthy 13-year- old girl was admitted in April 2022 with four weeks of fever, dry cough, loss of weight, followed by 1 week history of painful cervical lymphadenopathy and nonspecific maculopapular rash. She received her second dose of Covid 19 vaccine in January 2022. Unfortunately, she was diagnosed with CAT II, COVID 19 infection in March 2022. There was no history of allergy, recent traveling and cat scratch injury. Clinically there was no strawberry tongue, erythema of the lips, conjunctivitis or distal extremities changes to suggest Kawasaki disease. She was initially diagnosed with infection related lymphadenitis, treated with oral azithromycin for three days and intravenous ceftriazone for one week with no improvement. Her laboratory results showed hypochromic microcystic anaemia with leucopenia, raised inflammatory markers and lactate-dehydrogenese levels. Extensive workup for infection was unremarkable. Immunology test showed ANA, ANCA, ENA were negative with normal complements. Ultrasound abdomen was normal. Excisional lymph node biopsy revealed confluent areas of necrosis surrounded by histiocytes (CD68+) with absent of neutrophils. No granuloma or atypical lymphoid cells seen. Based on histopathology report, diagnosis of KFD was established. As she was not able tolerate orally, IV hydrocortisone was started and subsequently switched to oral prednisolone. She responded well to corticosteroids with fever subsided within a day and cervical lymphadenopathy reducing in size and resolved in one month. Prednisolone was able to taper off by two months. She showed complete recovery with no recurrence during follow-up. Conclusion(s): In persistent febrile painful lymphadenopathy, excision lymph node biopsy is essential to establish definite diagnosis. This case highlights the possible association between COVID-19 and KFD.

Post-pandemic healthcare for COVID-19 vaccine: Tissue-aware diagnosis of cervical lymphadenopathy via multi-modal ultrasound semantic segmentation.

With the widespread deployment of COVID-19 vaccines all around the world, billions of people have benefited from the vaccination and thereby avoiding infection. However, huge amount of clinical cases revealed diverse side effects of COVID-19 vaccines, among which cervical lymphadenopathy is one of the most frequent local reactions. Therefore, rapid detection of cervical lymph node (LN) is essential in terms of vaccine recipients' healthcare and avoidance of misdiagnosis in the post-pandemic era. This paper focuses on a novel deep learning-based framework for the rapid diagnosis of cervical lymphadenopathy towards COVID-19 vaccine recipients. Existing deep learning-based computer-aided diagnosis (CAD) methods for cervical LN enlargement mostly only depend on single modal images, e.g., grayscale ultrasound (US), color Doppler ultrasound, and CT, while failing to effectively integrate information from the multi-source medical images. Meanwhile, both the surrounding tissue objects of the cervical LNs and different regions inside the cervical LNs may imply valuable diagnostic knowledge which is pending for mining. In this paper, we propose an Tissue-Aware Cervical Lymph Node Diagnosis method (TACLND) via multi-modal ultrasound semantic segmentation. The method effectively integrates grayscale and color Doppler US images and realizes a pixel-level localization of different tissue objects, i.e., lymph, muscle, and blood vessels. With inter-tissue and intra-tissue attention mechanisms applied, our proposed method can enhance the implicit tissue-level diagnostic knowledge in both spatial and channel dimension, and realize diagnosis of cervical LN with normal, benign or malignant state. Extensive experiments conducted on our collected cervical LN US dataset demonstrate the effectiveness of our methods on both tissue detection and cervical lymphadenopathy diagnosis. Therefore, our proposed framework can guarantee efficient diagnosis for the vaccine recipients' cervical LN, and assist doctors to discriminate between COVID-related reactive lymphadenopathy and metastatic lymphadenopathy.

A Case of Acute Epiglottitis as a Presentation of Covid-19

INTRODUCTION: Acute epiglottitis is a potentially fatal condition due to high risk of respiratory failure from upper airway obstruction and resultant difficulty securing a patent airway. The etiology is most commonly infectious although noninfectious etiologies have been identified. We present a rare case of acute epiglottitis as a presentation of COVID-19. DESCRIPTION: A 37-year-old male with a past medical history of health presented due to acute throat pain with difficulty swallowing, and without dyspnea or cough. The patient took no medications or supplements, had no known sick contacts, chemical inhalation, recent travel, or any known allergies. He had never received vaccination for COVID-19. He occasionally smoked cigarettes and marijuana, and did not consume alcohol or use illicits. The patient was febrile and had a swollen, tender submandibular area and tender cervical lymphadenopathy. Solids, liquids, and saliva were difficult to swallow but he was in no respiratory distress. A CT of the neck showed the classic "thumb sign" of a thickened epiglottis as well as thickening of the laryngeal soft tissues. Visualized by flexible laryngoscopy, the epiglottis was erythematous and severely swollen;vocal cords functioned normally. He had neutrophilic predominant leukocytosis with monocytosis. A nasopharyngeal swab PCR was positive for SARS-CoV-2, however a broad work up for other viral and bacterial pathogens was negative. He was admitted to the intensive care unit and treated with broad spectrum antibiotics and high dose corticosteroids. After five days of monitoring, no intubation or airway intervention was needed. He improved as he tolerated a liquid diet and was discharged home with antibiotics. DISCUSSION: 18 cases of acute epiglottitis associated with COVID-19 have been reported, and the incidence is likely underestimated. The mechanism of epiglottitis in SARSCoV- 2 infection is poorly understood;whether directly caused by the virus versus by Increased susceptibility to bacterial superinfection. Clinicians should be aware of this condition due to the high risk of airway compromise and mortality without prompt diagnosis and appropriate management. Early involvement of skilled providers is critical as patients may require nasotracheal intubation, tracheostomy, or emergent cricothyrotomy.

Fever and rash: Do not MIS the diagnosis

Case description A 25-year-old patient was admitted for high fever (T: 39degreeC) since 1 week and a generalized maculopapular rash since 3 days. The eruption spread from his legs to his chest but spared his face. There were also papules on his hand palms while his foot soles were deeply erythematous. The patient described a sore throat, dry cough, slight retrosternal discomfort and muscle pain the week before. He also noticed a redness of his eyes since 2 days. There was no recent travelling, sexual intercourse and no animals at home. He was vaccinated against measles and COVID-19. His last proven COVID-19 infection dated from 8 months earlier. He was not taking medication and had no past medical history. Clinically, the patient had a generalized maculopapular eruption, multiple cervical lymphadenopathy, cheilitis, bilateral conjunctivitis and a hepatosplenomegaly. Labs showed a CRP at 312 mg/L, neutrophil count at 13.000/mm c, platelet count at 169.000/mm c, total bilirubin at 1,7 mg/dL (mostly direct), ASAT 100 U/L, ALAT 150 U/L, GGT 202 U/L and LDH 348 U/L. Creatinine level was 1,17 mg/dL. Troponine count was 187ng/L. D-dimers were elevated at 3069 ng/mL and ferritin level was also elevated at 3268 microgramme/L. There was a slight disturbance of INR (1,9). NT-ProBNP level was 1911 pg/mL. Urine analysis was normal and blood cultures were negative. Chest X-ray was negative. Abdominal ultrasound confirmed a hepatosplenomegaly. A transthoracic echocardiography showed an ejection fraction of 51% without signs of pericarditis. Serology for Hepatitis A, C, HIV, CMV, Syphilis, Mycoplasma were negative. The patient was immune for Hepatitis B and measles. PCR for measles was negative. He had already been in contact with Parvovirus B19 (IgG positive) and EBV (IgM and IgG positive with presence of anti EBNA). His serology for COVID-19 was positive with high levels of anti-nucleocapsid and anti-spike S1 antibodies but PCR on nasopharyngeal swab was negative. Autoimmune serology were negative (ANA, ANCA). We suspected the presence of a multisystem inflammatory syndrome of the adult (MIS-A) with cardiac involvement because of the Kawasaki-like dermatological presentation and the high inflammatory levels. We had no proof of a more recent SARS-CoV-2 infection than the infection of 8 months earlier. A cardiac monitoring was proposed and the patient was treated by intravenous immunoglobulin and corticosteroids. His general state improved rapidly and he was discharged after 10 days of hospitalization with a tapering dose of corticosteroid given orally. Conclusion(s): MIS-A is a novel syndrome secondary to SARS-Cov-2 infection, in the beginning mostly described in children (MIS-C) but also described in adults. There is fever, high inflammation, and severe illness with multisystem organ involvement (>=2). Cardiac involvement is present in most of the cases. Serology is often positive but PCR can be negative, suggesting the presence of a dysregulation of the immune system more than an acute infection. The mortality rate is 1-2% so physicians should be able to recognize this disease in order to give the best treatment.

Cervical and Axillary Lymphadenopathy after Administrating mRNA BNT-162b2 Vaccine against COVID-19: Clinical and Sonographic Findings, Outcomes of Short-term Follow-up

Introduction: Vaccination plays an important role in all strategic actions against the COVID-19 pandemic. Despite the high safety and efficacy of vaccination, side effects of the vaccines may also occur. The purpose of this study was to evaluate the clinical and sonographic findings and short-term results of cervical and axillary lymphadenopathy after the BNT-162b2 mRNA vaccine. Materials and Methods: The patients who received at least one dose of BNT-162b2 mRNA vaccine between July-September 2021 and were detected to have ipsilateral axillary and cervical lymphadenopathy related closely to the vaccination period, were included in the study. Clinical characteristics, sonographic findings of lymphadenopathies, and short-term results were analyzed retrospectively. Results: A total of 13 patients [six females (46.2%), seven males (53.8%)] were evaluated in the present study. Mean age of the patients was 41.9 years (min-max= 20-56). Median time-lapse between vaccination and presentation to hospital was six days, and seven (53.8%) patients presented with symptoms and findings after the first dose, and six patients (46.2%) after the second dose. Three (23.1%) axillary lymphadenopathies, and 10 (76.9%) cervical lymphadenopathies were detected. Sonographic examination revealed lymphadenopathies predominantly oval morphology (69.2%), asymmetric cortical thickening (61.5%), and hilar-type vascularization (69.2%). Mean time of regression was found 19.2 days (min-max= 10-35). Conclusion: Ipsilateral cervical and axillary lymphadenopathies may occur because of vaccines against COVID-19. The sonographic findings of these lymphadenopathies may not be distinguished clearly from malignant lymph nodes;and for this reason, close clinical and radiological follow-up would be appropriate to elucidate the process.

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

PEDIATRIC-TYPE FOLLICULAR LYMPHOMA - A NEW PARADIGM IN DIAGNOSIS?

Background: Recognized as an entity in the 2016 WHO Classification of Tumors of Hematopoietic and Lymphoid Tissues, Pediatric-Type Follicular Lymphoma (PTFL) is a rare nodular follicular lymphoma that affects primarily children and young adults. The clinical presentation is characterized by the sudden appearance of an isolated lymphadenopathy, with a predilection for the head and neck region, without systemic symptoms. The incidence is higher in men. It has an excellent prognosis with the excision of the affected ganglion. By definition, diagnosis is histological, immunocytochemical and molecular. There are no known risk factors or any described association with immunodeficiency or viral infections. Aims: We report two clinical cases. Methods: Case 1 - a previously healthy 18-year-old boy with an isolated, non-painful, cervical lymphadenopathy of approximately 20 mm, which was incidentally found. Case 2 - a 13-year-old boy without relevant personal history, who, after the second dose of vaccination against COVID-19, developed multiple adenomegalies that spontaneously regressed. However, one month later, a right submandibular adenomegaly appeared. It was analysed by ultrasound and was described as suspicious. In both cases, a fine-needle lymph node biopsy was performed for cytological diagnosis and material was sent for immunophenotyping by flow cytometry and molecular cytogenetics by FISH. Results: Immunophenotyping suggested a large-cell B-lymphoma with a phenotype compatible with Burkitt's Lymphoma (BL). In the cytology of both cases, the population observed was more consistent with diffuse large B cell lymphoma (DLBCL) or possibly high-grade follicular lymphoma (FL). In the FISH study, no rearrangements in the MYC, BCL2, BCL6 or IRF4 genes were detected in the samples of the two cases. The lymphadenopathies were excised with a probable diagnosis of PTFL or DLBCL. Histological examination confirmed the PTFL diagnosis. Summary/Conclusion: We did not find in the literature any reference to clear causal relationship between vaccination against COVID-19 and the onset of lymphoproliferative diseases. The cytological/immunophenotypic/molecular approach of this entity in both cases seems to define a characteristic pattern, which may eventually allow, in a first approach, to suspect this diagnosis. More extensive studies will be needed to establish the role of these methodologies in the diagnosis of this pathology.