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.

Adverse Effects of Covid-19 Vaccine, an Observational Studymainly Focusing on the Cutaneous Reactions in Both Genders

Objective: COVID-19 caused a deleterious impact on the health care system globally.The roll out of vaccines seems to be the only effective way to curtail the spread of disease.The purpose of this study is to assess the dermatological adverse effect of post COVID-19vaccination on a gender basis. Methodology: This was an observational,cross-sectional,questionnaire-based survey conducted in Pakistan.The sample comprises 518 participants. The questionnaire was self-designed. The trial lasted six months, from August 1, 2022, until January 31, 2023. We used a non-probability sampling technique.Dermatological adverse effects like burning pain, redness, rashes, and lymphadenopathy at the injection site were recorded.Fever was also noted. All the participants have received booster shots or double doses of any one of CCOVID-19 vaccines, such as AstraZeneca, Pfizer, Sinovac, Sinopharm,Pakvac, etc. A p-value of less than 0.05 was considered statistically significant.Qualitative data was reported as frequency and percentage, and quantitativedata was reported as standard deviation and mean. Result(s): The study included 518 subjects, of whom 262 were males and 256 were females. The mean age of male is 42.70+/-14.05 years and female is 39.04+/-14.6years with a significant difference observed between them (p=0.004). The most common complaint among dermatological adverse effects after first was pain. 106(40.5%) male and 132(51.6%) female reported painwith a significant difference observed between them (p=0.011) followed by swelling which was reported by 92(35.1%) males and 120(46.9%) females with a significant difference observed between them (p=0.006).Burning was reported in 92(35.1%) male and 148(57.8%) female with a significant difference observed between them(p<0.001). Fever was also quite commonly reported in both male 116(44.3%) and female 178(69.5%) with significantdifference observed between them (p<0.001),Likewise post 2nd dose of vaccination, pain was most commonly noted in 90(34.4%) male and female 124(48.4%) female with significant difference observed between them (p=0.001). Moreover, burning was reported by 80(30.5%) malesand 132(51.6%) females with a significant difference observed between them (p<0.001). rashes were reported by76(29.0%) males and 100(39.1%) females with a significant difference observed between them (p=0.016), lymphadenopathy was also significantly associated with genders, (p<0.001). Conclusion(s): This study concluded that the burning pain,redness,rashes,and lymphadenopathywere the most prevalent side effects in male and female post 1st and 2ndCOVID-19 vaccination.Furthermore fever was also reported in majority of subjects.In addition to this higher percentage of side effects were recorded in females as comparedto males.Copyright © 2023 Lahore Medical And Dental College. All rights reserved.

A Case of a Relapsing Remitting Macrophage Activating Syndrome After Covid-19 Vaccine In a Teenager with UNC13D Heterozygous Variant of Uncertain Significance

Introduction: Macrophage activation syndrome (MAS) is a severe hyper inflammatory condition caused by the over-activation and proliferation of T cells, NK cells and macrophages. It is often associated with complications of rheumatic/immune diseases. We present a case of a 15-year-old female who experiences recurrent episodes of MAS without any known definitive underlying etiology. Case Presentation: A 15-year-old previously healthy female developed fatigue, fevers, myalgia, chest pain, splenomegaly and lymphadenopathy 10 days after receiving her first Pfizer COVID-19 vaccine. Her symptoms recurred 10 days after receiving the second dose. Her myocarditis, MIS-C, and infectious work up was negative except for positive EBV IgG. Laboratory studies revealed anemia, hypertriglyceridemia, hypofibrinogenemia, and hyperferritinemia. She initially responded to decadron;however, her symptoms recurred with steroid taper. Bone marrow biopsy revealed hemophagocytosis. Whole exome sequencing (WES) revealed a heterozygous variant of uncertain significance in UNC13D c.962C>A (p.Thr321Asn). She had multiple re-admissions with significantly elevated inflammatory markers, including extremely high IL2-R, IL-18 and CXCL9. Each episode was complicated by an acute viral infection. She responds to high dose steroids, anti-IL-1, and JAK inhibitors. Nonetheless, it has been difficult to wean decadron without triggering a flare. She continues to require increasing doses of baricitinib. Discussion(s): MAS may be seen as a complication of rheumatic diseases, as well as inborn errors of immunity. However, none of these conditions have been diagnosed in this patient despite extensive testing, including WES. The degree of her immune dysregulation has been very severe making her disease process unpredictable and extremely difficult to control. She has frequent flares precipitated by viral infections or attempts at adjusting her immunomodulators. Weaning her medications has been challenging as she continues to require increasing doses of baricitinib and corticosteroids. The UNC13D gene is associated with autosomal recessive familial hemophagocytic lymphohistiocytosis type 3 (FHL3). Our patient is heterozygous for an UNC13D variant of uncertain significance. Additional genetic inquiries with whole genome sequencing to help elucidate the underlying etiology of her severe condition is being conducted. We hypothesize she developed MAS due to a combination of genetic predisposition, prior EBV infection, and immune stress associated with the COVID-19 vaccine. [Formula presented] [Formula presented] [Formula presented]Copyright © 2023 Elsevier Inc.

Case Report of Succesful Multidisiplinary Treatment for Patient with Metastatic Lung Cancer and Relapsed/Refractory Chronic Lymphatic Leukemia/Small Lymphocytic Lymphoma

Introduction: Croatian National Cancer Registry of Croatian Institute for Public Health reported that in year 2020 lung cancer was the second most common cancer site diagnosed in men with 16% and the third most common in women with 10% incidence among all cancer sites. Unfortunatelly lung cancer has the highest mortality in both men and women. Haematological malignancies had 7% share in all malignancies in both male and female cances cases. In 2020 190 newly diagnosed cases of lymphatic leukemia in men and 128 cases in women were reporeted, meaning 1.5 and 1.2% of all malignancies, respectively. Chronic lymphatic leukemia (CLL) is an advanced age disease and incidence increases with age. Impaired immunity, T and B cell dysfunction in CLL, chromosomal aberations, long-term immunosuppressive therapy and genetic factors can all cause secondary malignancies. Co- occurence of solid tumors and CLL is very rare. Although patiens with CLL have an increased risk of developing second primary malignancies including lung carcinoma, the data about their clinical outcomes are lacking. Parekh et al. retrospectively analyzed patients with simultaneous CLL and lung carcinoma over a 20-year period, and they found that ~2% of patients with CLL actually developed lung carcinoma. The authors claimed that up to 38% of patients will also develop a third neoplasm more likely of the skin (melanoma and basal cell carcinoma), larynx (laryngeal carcinoma) or colon. Currently there are no specific guidelines for concurrent CLL and non-small cell lung carcinoma (NSCLC) treatment. Usually, when the tumors are diagnosed simultaneously, treatment is based to target the most aggressive malignancy, as the clinical outcomes depend on the response of the tumor with the poorest prognosis. For this reason, a multidisciplinary approach is mandatory. Case report: A patient with history of coronary heart disease, myocardial infarction and paroxysmal atrial fibrillation was diagnosed in 2019 (at the age of 71) with B chronic lymphocytic leukemia with bulky tumor (inguinal lymph nodes 8x5 cm), stage B according to Binet, intermediate risk. He was treated with 6 cycles of chemoimmunotherapy (rituximab/cyclofosfamid/fludarabine). In 10/2019 remission was confirmed, but MSCT described tumor in the posterior segment of upper right lung lobe measuring 20x17 mm and bilateral metastases up to 11 mm. Bronchoscopy and biopsy were performed, and EGFR neg, ALK neg, ROS 1 neg, PD-L1>50% adenocarcinoma was confirmed. He was referred to Clinical Hospital Center Osijek where monotherapy with pembrolizumab in a standard dose of 200 mg intravenously was started in 01/2020. Partial remission was confirmed in October 2020. Immunotherapy was discontinued due to development of pneumonitis, dysphagia and severe weight loss (20kg), but without radiologically confirmed disease progression. At that time he was referred to our hospital for further treatment. Gastroscopy has shown erosive gastritis with active duodenal ulcus, Forrest III. Supportive therapy and proton pump inhibitor were introduced. After complete regression of pneumonitis, improvement of general condition and resolution of dysphagia, no signs of lung cancer progression were found and pembrolizumab was reintroduced in 12/2021. Hypothyroidism was diagnosed in 01/2021 and levothyroxine replacement ther apy was started. In 03/2021 he underwent surgical removal of basal cell carcinoma of skin on the right temporal region with lobe reconstruction. From 02/2021, when pembrolizumab was reintroduced, regression in tumor size was continously confirmed with complete recovery of general condition. He was hospitalized for COVID 19 infection in 09/2021, and due to complications pembrolizumab was discontinued till 11/2021. Lung cancer immunotherapy proceeded till 11/2022, when Multidisciplinary team decided to finish pembrolizumab because of CLL relapse. CLL was in remission till August 2022 when due to B symptoms, lymphcytosis, anemia and generalized lymphadenopathy, hematological workup including biopsy of cervical lymph node was performed and CLL/SLL relapse was confirmed. Initially chlorambucil was introduced, but disease was refractory. Based on cytogenetic test results (IGHV unmutated, negative TP53) and due to cardiovascular comorbidity (contraindication for BTK inhibitors) venetoclax and rituximab were started in 01/2023. After just 1 cycle of treatment normal blood count as well as regression of B symptoms and peripheral lymphadenopathy occured, indicating the probability of complete disease remission. In our patient with metastatic lung adenocarcinoma excellent disease control is achieved during 41 month of treatment in first line setting. Furthermore, relapsed/refractory CLL/SLL is currently in confirmed remission. Conclusion(s): Successful treatment of patients with multiple primary malignancies is based on multidisciplinarity, early recognition and management of side effects, treatment of comorbidities with the aim of prolonging life, controlling symptoms of disease and preserving quality of life.

EBV-related lymphoproliferative disease in Wiskott-Aldrich Syndrome patient

Case: Wiskott-Aldrich Syndrome (WAS) is a rare X-linked inborn error of immunity caused by mutations in the WAS gene. It is classically characterized by immunodeficiency, eczema, and micro-thrombocytopenia. It has been known since the 1960s that patients with WAS have an increased risk of lymphoproliferative disease though the exact incidence remains unknown in the American population. Limited case reports have discussed EBV-related lymphoproliferative disease in patients with WAS. We present a case of a 9-year-old boy with known WAS complicated by eczematous rash, thrombocytopenia, recurrent ear infections, and monoclonal gammopathy who was found to have submandibular EBV-associated lymphoid hyperplasia with associated lung and retroperitoneal lymphadenopathy. Family had been offered treatment with hematopoietic stem cell transplant but declined multiple times in the past. Earlier in the year, he presented with possible MIS-C with negative SARS-CoV-2 PCR. He presented to our hospital with mastoiditis and lymphadenopathy. Physical examination showed severe eczema on hands and tender right mastoid. Laboratory evaluation showed thrombocytopenia, elevated IgG of 6290, IgA of 744, IgE of 827, low IgM of 41, and 14% response to pneumococcal titers. He was empirically treated with intravenous antibiotics. ENT performed right postauricular incision and drainage and the culture grew Hemophilus influenza. Throughout his hospital stay, his submandibular lymphadenopathy became more prominent despite treatment. Core needle biopsy of right submandibular lymph node was suggestive of EBV-associated lymphoid hyperplasia. EBV PCR and antibodies were both positive. CT chest, abdomen, and pelvis revealed multifocal pulmonary lymphadenopathy and a diffuse, bilateral nodularity as well as retroperitoneal and mesenteric lymphadenopathy. He was given four doses of weekly Rituximab, which successfully decreased EBV viremia below linear detectability. Immunoglobulin replacement therapy (IgRT) was initiated. Bronchoalveolar lavage and lung biopsy were performed and are results are currently pending. Discussion(s): We present a case of a 9-year-old boy with known WAS awaiting transplant who was found to have submandibular EBV-associated lymphoid hyperplasia with associated lung and retroperitoneal lymphadenopathy. While lymphoproliferative disease is a known complication of WAS, EBV-related lymphoproliferative disease in WAS patients has only been reported as case reports and remains a rare but known complication of patient with WAS.Copyright © 2023 Elsevier Inc.

Systemic and Local Disease Manifestations Following COVID-19 Vaccination

Introduction/Objective COVID-19 vaccine-related lymphadenopathy, particularly in the ipsilateral axilla, is a relatively well-known side effect of mRNA vaccines with many reports in radiology, but less is known regarding histopathology and additional sites of lymphadenopathy, as well as other localized potential vaccine-related mass manifestations. In addition to a case of minimal change disease, we report two cases here with associated systemic and local pathologic changes related to COVID-19 vaccination. Methods/Case Report In case #1, a 17-year-old male presented with a 2.4 cm left postauricular mass. He had originally noticed the mass six months prior and thought that it had recently been growing. The mass was soft, nonfluctuant, and nontender to palpation. Given the risk of malignancy, a resection was performed. Histology showed an enlarged lymph node composed of mixed inflammatory cell components consistent with lymphoid hyperplasia and no evidence of malignancy. On further chart review, the patient had received his second COVID-19 vaccination just prior to noticing the mass enlarging. A SARS-CoV-2 Anti-Spike IgG assay was as high as 24,396 AU/ml, suggesting that this benign lymphadenopathy was most likely related to his vaccination. For case #2, a 47-year-old male developed a painless right deltoid mass shortly after receiving his vaccination at the same area that subsequently increased in size over seven months to 6.5 cm. Imaging showed a heterogeneous mass within the deltoid muscle concerning for malignancy and a biopsy was performed. Sections showed wavy, bland spindle cells with nuclei staining diffusely positive for beta-catenin, consistent with fibromatosis at his vaccination site. Results (if a Case Study enter NA) NA. Conclusion In summary, these case reports show potential systemic and local reactive effects in response to COVID-19 vaccination.

Development of warm autoimmune hemolytic anemia after initiation of Ruxolitinib in a patient with STAT1 gain-of-function mutation

Introduction: STAT1 gain-of-function (GOF) disease is associated with chronic mucocutaneous candidiasis (CMC) and a broad spectrum of infectious, inflammatory, and vascular manifestations. The Janus Kinase inhibitor ruxolitinib has been used successfully for CMC and autoimmune phenomena. We describe a case of warm autoimmune hemolytic anemia (WAIHA) in a patient with STAT1 GOF disease after initiating ruxolitinib. Case report: A 36-year-old man with STAT1 c.850G>A (p.Glu284Lys) mutation presented with CMC as well as recurrent viral and bacterial infections, lymphadenopathy, enteritis, nodular regenerative hyperplasia (NRH) and splenomegaly. Immune workup confirmed a combined immunodeficiency with hypogammaglobulinemia and T-cell lymphopenia. Ruxolitinib was initiated at 5 mg twice daily (due to pre-existing thrombocytopenia) with up titration over 3 months to 20 mg twice daily. He improved with weight gain, increased energy, resolution of chronic anemia, and improved lymphadenopathy and splenomegaly on imaging. Serum CXCL9 only minimally decreased from 4660 pg/ml to 3990 pg/ml. Soon after reaching ruxolitinib 20 mg twice daily, he developed JC viremia, prompting dose reduction to 15 mg BID. Within two weeks, he developed a non-COVID upper respiratory tract infection followed by fatigue, shortness of breath with ambulation, and dark urine. Emergency evaluation revealed warm antibody positive hemolytic anemia with a hemoglobin of 5 g/dL, and worsened thrombocytopenia. He was treated with blood transfusions, pulse steroids, and high-dose IVIG with stabilization but continued hemolysis. Due to the JC viremia, there was concern to give rituximab with increased PML risk. Bone marrow showed trilineage hematopoiesis, a mild increase in megakaryocytes and RBC precursors, and a loss of B-cell progenitors with retention of mature B cells. His B and T lymphocyte numbers had increased since prior to ruxolitinib, with a predominance of Tfh1-cells (58.7% of total Tfh-cells). He was started on sirolimus with a slow taper of prednisone with continued stable hemoglobin and platelets, and resolution of hemolysis after 3 months. Conclusion(s): To our knowledge, this is the first case of a STAT1 GOF patient developing WAIHA while receiving ruxolitinib therapy. Treatment choices were complicated by the risks of PML. Sirolimus combined with ruxolitinib allowed wean of corticosteroid and subsequent resolution of hemolysis.Copyright © 2023 Elsevier Inc.

Lymphoproliferation- a clue towards an underlying monogenic disorder of immune dysregulation- a retrospective analysis from a single center in India

Background: Lymphoproliferation is the persistent proliferation of lymphoid cells and it's incidence in inborn errors of immunity varies from 0.7 to 18%. Material(s) and Method(s): This is a retrospective analysis of patients referred to the department of Immunology, B. J. Wadia Hospital for Children, Mumbai between March 2017 to December 2022. Inclusion criteria consisted of 3 months duration of significant lymphadenopathy and/or splenomegaly or history of lymphoma. The clinical characteristics, laboratory and molecular findings of the included patients were analyzed. Result(s): A total of 66 patients were included. There was a male preponderance with male:female ratio of 25:8. Median age of onset of lymphoproliferation was 4.75 years(Range 1 year to 60 years). Splenomegaly was seen in 75%. Infections included recurrent pneumonia (14/66), recurrent ear infections(5/66), COVID(4/66), one episode of pneumonia(6/66), herpes zoster(3/66), recurrent subcutaneous abscess (3/66), abdominal koch(3/66), chronic sinusitis(2/66), dermatophytosis(2/66), esophageal candidiasis(2/66), recurrent malaria(1/66), recurrent varicella(1/66), cryptococcal meningitis(1/66), gram negative sepsis(1/66), BCG adenitis(1/66), pseudomonas osteomyelitis(1/66), impetigo (1/66), pseudomonas urinary tract infection (1/66), chicken pox(1/66), herpes keratitis(1/66), dengue(1/66), Other manifestations included Evans plus phenotype(10/66), Evans phenotype(8/66), Autoimmune hemolytic anemia(5/66), bronchiectasis(5/66), Type 1 diabetes(3/66), hyper reactive airway disease(2/66), inflammatory bowel disease(4/66), autoimmune thrombocytopenia(2/66), stroke(3/66), hemophagocytic lymphohistiocytosis(2/66), hypertriglyceridemia(2/66), hypothyroidism(2/66), celiac disease(1/66), Type 2 diabetes(1/66), autoimmune encephalitis(1/66), autoimmune hepatitis(2/66), anti-parietal cell antibody(1/66), arthritis(1/66), autoimmune enteropathy(1/66), systemic lupus erythromatosus(1/66), primary biliary cirrhosis requiring liver transplant(1/66), nephrotic syndrome(1/66), lymphoedema(1/66), hypersplenism(1/66), recurrent oral ulcers(1/66), gout(1/66), dermatitis(1/66), ovarian teratoma(1/66), alopecia areata(1/66). Hodgkin's lymphoma(HL) was the most common malignancy(9/66), followed by non Hodgkin lymphoma(NHL)(6/66), transformation from NHL to HL(1/66), Burkitt to T-cell lymphoma(1/66), HL to DLBCL(1/66), HL to anaplastic T-cell lymphoma(1/66). EBV driven lymphoproliferation was seen in biopsy of21/66. Genetic testing showed mutations in LRBA(11/66), PIK3CD(5/66), CTLA4(3/66), TET2(2/66), IL2RA (1/66), IL12RB1(1/66), BACH2(1/66), PRKCD(1/66), TNFSFR13B(1/66), TNFAIP3(1/66), FAS(2/66), FASL(1/66), Caspase8(1/66), CARD11(1/66), RTEL1(1/66), AICD(1/66), PIK3R1(1/66), IKBKB(1/66). Treatment included IVIG, chemotherapy, rituximab, sirolimus, abatacept, HSCT. Conclusion(s): All children with persistent lymphoproliferation, with or without autoimmunity and/or infections should be worked up for an underlying monogenic disorder of immune dysregulation. Lymphomas presenting at abnormal site and/or age, relapse and EBV driven lymphomas require further evaluation. Presence of monogenic cause helps in providing targeted therapy.Copyright © 2023 Elsevier Inc.

Monkeypox (mpox) is a new problem on a global scale for humanity.

Purpose - to draw attention to an infection that was little known, but has now become a global problem for society;to familiarize readers with the peculiarities of the 2022 monkeypox outbreak and to increase the level of alertness of doctors to this disease. Monkeypox is a global problem because the disease is spreading rapidly, covering 111 countries. Three cases were diagnosed in Ukraine. It is predominantly a self-limited infection, but there are severe and deadly complications. The lethality of this disease ranges from 0% to 11%. The course of the disease is more severe in children and people with reduced immunity. Vertical transmission of the virus from mother to child is possible, resulting in congenital monkeypox. Monkeypox is a zoonotic disease and its natural reservoir is not exactly known, but rodents are most likely to act. In most cases, person-to-person transmission of the virus occurs through close skin to skin contact, often during sexual intercourse. At the beginning of the outbreak 98% of cases of disease were was diagnosed in homo- and bisexuals. Airborne transmission is also possible. Infection is possible through close contact with infectious skin lesions. Clinically, the initial period resembles influenza, but lymphadenopathy is characteristic, which is considered a pathognomonic symptom of mpox. The rash is similar to that of chickenpox, but with more prevalent location on palms and soles than in chickenpox. In the presence of a vesicular rash in a patient, it is necessary to exclude monkeypox. PCR diagnostics of the virus in samples of vesicles or crusts has the greatest diagnostic value. Hygienic skin care is important. Antiviral drugs (tecovirimat, brincidofovir) are recommended only in severe cases. To reduce the spread of infection, international rules apply as for other infections, such as COVID-19. The monkeypox virus vaccine is recommended primarily for groups at risk of infection, including medical personnel who may come into contact with the patient or samples for laboratory testing. Being aware for this infection, following international health regulations, it is possible to prevent the further spread of monkeypox.Copyright © 2023 Tomsk State University. All rights reserved.

The Compatibility of Chest CT Scan with RT-PCR in Suspected COVID-19 Patients

BACKGROUND: Thoracic computed tomography (CT) scan plays a role in detecting and assessing the progression of COVID-19. It can evaluate the response to the therapy given. In diagnosis, the CT scan of the chest may complement the limitations of reverse transcription polymerase chain reaction (RT-PCR). Several recent studies have discussed the importance of CT scans in COVID-19 patients with false-negative RT-PCR results. The sensitivity of chest CT scan in the diagnosis of COVID-19 is reportedly around 98%. AIM: This study aimed to determine the compatibility of CT scan of the thorax with RT-PCR in suspected COVID-19 patients. MATERIALS AND METHODS: This research was conducted in the Radiology Department of the Wahidin Sudirohusodo Hospital Makassar from April to December 2020 with 350 patients. The method used was a 2 x 2 table diagnostic test. RESULT(S): The study included 188 male patients (53.7%) and 162 female patients (46.2%). The most common age group was 46-65 years (35.4%). The most common types of lesions were ground-glass opacity (163 cases), consolidation (128 cases), and fibrosis (124 cases), mostly found in the inferior lobe with a predominantly peripheral or subpleural distribution. The sensitivity of the CT scan to the PCR examination was 86%, and the specificity was 91%. CONCLUSION(S): Thoracic CT scan was a good modality in establishing the diagnosis of COVID-19. CT scan of the chest with abnormalities could confirm the diagnosis in 88% of cases based on RT-PCR examination. It excluded the diagnosis in 91% based on the RT-PCR examination. The accuracy of the thoracic CT scan was 88% with RT-PCR as the reference value.Copyright © 2023 Sri Asriyani, Albert Alexander Alfonso, Mirna Muis, Andi Alfian Zainuddin, Irawaty Djaharuddin, Muhammad Ilyas.

Reactive lymphadenopathy due to Pfizer-BioNTech COVID-19 vaccine: a case report / Linfadenopatía reactiva por vacuna Pfizer-BioNTech para COVID-19: reporte de un caso

Uno de los efectos secundarios encontrados en pacientes con antecedente de vacunación por COVID-19, especialmente con la vacuna Pfizer-BioNTech, es la aparición de múltiples adenopatías hiperplásicas, principalmente en los ganglios linfáticos axilares, supraclaviculares e infraclaviculares ipsilaterales al sitio de vacunación. Presentamos el caso de una paciente femenina de 33 años, con aparición de masa dolorosa supraclavicular izquierda, quien una semana antes había sido vacunada con la primera dosis de la vacuna Pfizer-BioNTech en región deltoidea izquierda. Los hallazgos citológicos fueron sugestivos de una enfermedad linfoproliferativa, y el estudio histopatológico reveló linfadenopatía reactiva con proliferación de inmunoblastos B activados, secundaria a la vacunación contra COVID-19. Aportamos a la literatura con la caracterización de los hallazgos histopatológicos de la linfadenopatía posvacunación contra COVID-19. Es importante que los médicos tratantes y radiólogos estén familiarizados con este diagnóstico diferencial, para brindar recomendaciones adecuadas basadas en un seguimiento a corto plazo, en lugar de realizar biopsias, intervenciones y conductas inmediatas innecesarias en el manejo de los pacientes

One of the side effects found in patients with a history of vaccination for COVID-19, especially with the Pfizer-BioNTech vaccine, is the appearance of multiple hyperplastic adenopathies, mainly axillary, supraclavicular and infraclavicular lymph nodes ipsilateral to the vaccination site. We present the case of a 33-year-old female patient, with the appearance of a painful left supraclavicular mass, who was vaccinated a week earlier with the first dose of the Pfizer-BioNTech vaccine in the left deltoid region. The cytological findings were suggestive of a lymphoproliferative disease, and the histopathological study revealed reactive lymphadenopathy with proliferation of activated B immunoblasts, secondary to vaccination against COVID-19. We contribute to the literature with the characterization of the histopathological findings of COVID-19 post-vaccination lymphadenopathy. It is important for treating physicians and radiologists to be familiar with this differential diagnosis, in order to provide appropriate recommendations based on short-term follow-up, instead of performing unnecessary immediate biopsies or interventions in patient management.

Anaplastic Large-Cell Lymphoma (ALCL) First Manifested as a Rapidly Progressive Acute Respiratory Failure (RF): Lymph Node Biopsy Negative Presentation Posing a Diagnostic Challenge.

Anaplastic large-cell lymphoma (ALCL) is an aggressive subtype of non-Hodgkin lymphoma. There are two forms of ALCL: primary and secondary. Primary can be systemic, affecting multiple organs, or cutaneous, affecting mainly the skin. A secondary form occurs when another lymphoma undergoes an anaplastic transformation. ALCL rarely presents as initial symptoms of respiratory failure. In most of these situations, the trachea or bronchial involved with an obstruction was present. We present an unusual case of ALCL where the patient rapidly progressed to acute hypoxic respiratory failure with a patent bronchus and trachea. Unfortunately, the patient rapidly deteriorated and died before diagnosis. Only upon at autopsy, it was found that his lung parenchyma was diffusely involved with ALCL. The autopsy report showed that the patient had CD-30 anaplastic lymphoma kinase (ALK)-negative ALCL diffusely involving all lung fields.

[18F]FES PET Resolves the Diagnostic Dilemma of COVID-19-Vaccine-Associated Hypermetabolic Lymphadenopathy in ER-Positive Breast Cancer.

Coronavirus disease (COVID-19) vaccination is known to cause a diagnostic dilemma due to false-positive findings on [18F]FDG PET in vaccine-associated hypermetabolic lymphadenopathy. We present two case reports of women with estrogen-receptor (ER)-positive cancer of the breast who were vaccinated for COVID-19 in the deltoid muscle. [18F]FDG positron emission tomography (PET) demonstrated primary breast cancer and multiple axillary lymph nodes with increased [18F]FDG uptake, diagnosed as vaccine-associated [18F]FDG-avid lymph nodes. Subsequent [18F]FES PET revealed single axillary lymph node metastasis in the vaccine-associated [18F]FDG-avid lymph nodes. To the best of our knowledge, this is the first study showing the usefulness of [18F]FES PET in diagnosing axillary lymph node metastasis in COVID-19-vaccinated patients harboring ER-positive breast cancer. Thus, [18F]FES PET has potential applications in the detection of true-positive metastatic lymph nodes in patients with ER-positive breast cancer regardless of the ipsilateral or contralateral side, who have received COVID-19 vaccination.

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.

A Diagnostically Challenging Case of Primary Pancreatic Lymphoma

Introduction: Primary Pancreatic Lymphoma (PPL) is the exceedingly rare instance of extranodal Non-Hodgkin's Lymphoma developing mainly in the pancreas.We report a diagnostically challenging case of a patient presenting with a rapidly growing pancreatic mass, found to have PPL. Case Description/Methods: A 48-year-old female with past history of tobacco use presented with several months of cramping abdominal pain following COVID-19 infection. She denied weight loss, fevers, or night sweats. Her physical exam, CBC, CMP, lipase, LDH, and CA 19-9 were unremarkable. An abdominal ultrasound revealed a 2.8 x 1.9 x 3 cm cystic mass of the pancreatic head, most congruent with a pseudocyst. Worsening abdominal pain prompted repeat ultrasound one month later, which showed a doubling in size. Endoscopic ultrasound (EUS) with fine needle aspiration of the cystic mass and surrounding lymph nodes yielded cystic contents and reactive lymphadenopathy. Two months later, her abdominal pain worsened and repeat imaging showed further doubling in size with encasement of the celiac plexus. A second FNA performed via EUS redemonstrated cystic contents. An ultrasound-guided core needle biopsy of the mass revealed necrotic CD301 diffuse large B cell lymphoma (DLBCL). PET scan was suggestive of stage IV PPL (Figure). Imaging also identified an inguinal lymph node that returned as CD101 BCL61 high grade follicular lymphoma, which was thought to be a distinct lesion. She was started on R-CHOP. Her clinical course was complicated by the formation and subsequent rupture of a splenic artery pseudoaneurysm, gastrointestinal bleeding, anuric kidney injury, and intestinal ischemia. She ultimately transitioned to comfort care. Discussion(s): Primary pancreatic lymphoma comprises 0.6% of extranodal lymphomas and 0.2% of primary pancreatic tumors. The clinical presentation is often vague and includes abdominal pain, B symptoms, jaundice, or bowel obstruction. The diagnostic criteria according to the WHO requires that the (1) majority of tumor burden be localized to the pancreas and (2) existing nearby and distant lymph node involvement should be secondary to pancreatic presentation. A biopsy is required to diagnose PPL, which is histologically most often DLBCL. Our case highlights the challenges associated with diagnosing PPL despite two EUS with FNA. Although rare, one should proceed with a high index of suspicion for PPL in any patient presenting with a rapidly enlarging pancreatic mass.

Hypermetabolic ipsilateral supraclavicular and axillary lymphadenopathy: Optimal time point for performing 18F-FDG PET/CT after COVID-19 vaccination

Ziel/Aim We aimed at evaluating the incidence of SARS-CoV2 vaccine-related axillary and supraclavicular hypermetabolic lymphadenopathy (HLA) and evaluate which timepoint produces the least number of false-positive findings in HLA. Methodik/Methods For this retrospective, multi-center imaging study patients with any form of SARS-CoV2 vaccination prior to 18F-FDG-PET/CT between January 2021 and December 2021 were included. Patients were divided into six groups according to the timepoint of vaccination prior to 18F-FDG-PET/CT imaging: Group 1 (0-6 Days), Group 2 (7-13 Days), Group 3 (14-20 Days), Group 4 (21-27 Days), Group 5 (28-34 Days) and Group 6 (35-80 Days). As reference SUVmax of mediastinal blood pool (MBP) and SUVmax contralateral reference lymph node (RL) were determined. For each group, the following parameters were assessed. A) absolute SUVmax of HLA B) incidence of HLA [defined as the ratio of SUVmaxHLA/ SUVmax Mediastinal Blood Pool (rHLA/MBP)] greater than 1,5 C) rHLA/MBP D) ratio SUVmax HLA vs. SUVmax contralateral reference lymph node (rHLA/RL). Ergebnisse/Results HLA showed the highest incidence in Group 1(day 0-6) 16/23 (70 %). Similarly, SUVmax HLA and rHLA/MBP were highest in this group, SUVmax 4.97 +/- 4.1 and 2.58 +/- 2.1 respectively. The incidence of HLA, SUVmax HLA, and rHLA/MBP were higher in Group 3 (14-20 days) than in Group 2 (7- 13days);57 % vs 44 %;5.05 +/- 4.33vs 3.9 +/- 2.81 (p = 0.723 and 2.32 +/- 1.8 vs 1.83 +/- 1.38(p = 0.788). All parameters for HLA dropped markedly after at least 21 days of vaccination. There were no significant differences in SUVmax HLA, rHLA/MBP and rHLA/RL in group 4 (21-27 days), group 5 (28-34 days) and group 6 (35-80 Days). [1] Schlussfolgerungen/Conclusions It is crucial for diagnostic physicians to assess the recent history of COVID-19 vaccination prior to FDG-PET/CT scan to reduce the risk of false-positive calls. If feasible, FDG PET should be postponed by at least 3 weeks after SARS-CoV2 vaccination especially if an accurate evaluation of axillary status is required.