ABSTRACT
SESSION TITLE: Unique Inflammatory and Autoimmune Complications of COVID-19 Infections SESSION TYPE: Rapid Fire Case Reports PRESENTED ON: 10/19/2022 12:45 pm - 1:45 pm INTRODUCTION: Coronavirus disease 2019 (COVID-19) can manifest as a severe immunologic syndrome known as hemophagocytic lymphohistiocytosis (HLH). HLH is a hyper-inflammatory state with a lethal mortality rate, especially when discovered late in the disease process. The optimal timely approach to diagnosis and treatment of secondary HLH in COVID-19 is unclear, however, risk stratification with Hscore using biomarkers can be useful to increase confidence in an HLH diagnosis. CASE PRESENTATION: A 36-year-old morbidly obese male with a history of well controlled mild intermittent asthma presented to the hospital complaining of a one week history of dyspnea and cough after failing outpatient COVID-19 treatment. Upon arrival, he was hypoxic on room air and was placed on non-invasive ventilation. He unfortunately decompensated further and was transferred to the intensive care unit where he was intubated for severe hypoxia and increased work of breathing. His course was complicated by superimposed bacterial pneumonia, vasopressor dependent septic shock, and anuric acute kidney injury requiring continuous renal replacement therapy. He remained profoundly hypoxic despite rescue therapy with multiple sessions of prone ventilation. On hospital day seventeen his platelets declined acutely and a serotonin release assay confirmed heparin-induced thrombocytopenia. His clinical status remained tenuous into the third week of admission. Notably, he developed persistent fever with associated bicytopenia and elevated lactate dehydrogenase, D-dimer, fibrinogen, triglycerides, and aspartate aminotransferase. His calculated Hscore was 189. Hematology recommended initiating HLH therapy with daily dexamethasone and etoposide, however the latter was held due to the patient's rapid hemodynamic decline. The patient succumbed to illness after a twenty-day hospitalization. His HLH was confirmed with a positive postmortem soluble-IL-2-receptor test. DISCUSSION: Proposals of routine HLH screening in critically ill patients are endorsed to promote early detection of this morbid condition. Calculating Hscore using vital signs, imaging, laboratory tests, and patient history can guide suspicion of diagnosis, since HLH-specific markers are often not feasible. Hscores more than 169 correspond to 93% sensitivity and 86% specificity in HLH diagnosis. Immunosuppression is standard therapy with hematology guidance due to the complex pathophysiology and limited research. CONCLUSIONS: This case emphasizes the importance of understanding the relationship between COVID-19 and secondary HLH. A timely diagnosis is vital in order to attempt to effectively treat a syndrome that carries a 65% mortality rate. Reference #1: Dimopoulos G, Mast Q. de, Markou N, et al. Favorable Anakinra responses in severe COVID-19 patients with secondary hemophagocytic lymphohistiocytosis. Cell Host Microbe 2020;doi: 10.1016/j.chom.2020.05.007. PubMed PMID: 32411313. Reference #2: Bauchmuller K, Manson JJ, Tattersall R, et al. Haemophagocytic lymphohistiocytosis in adult critical care. J Intensive Care Soc 2020;21:256–68. Reference #3: Schnaubelt, Sebastian MDa,∗;Tihanyi, Daniel MDb;Strassl, Robert MDc;Schmidt, Ralf MDc;Anders, Sonja MDb;Laggner, Anton N. MDa;Agis, Hermine MDd;Domanovits, Hans MDa Hemophagocytic lymphohistiocytosis in COVID-19, Medicine: March 26, 2021 - Volume 100 - Issue 12 - p e25170 doi: 10.1097/MD.0000000000025170 DISCLOSURES: No relevant relationships by Kristina Catania No relevant relationships by Katie Kennedy No relevant relationships by Josef Kinderwater No relevant relationships by MaryKate Kratzer no disclosure submitted for Ogugua Obi;
ABSTRACT
Airway abnormalities may be due to a multitude of conditions. Symptoms do not occur until there is very significant narrowing, and therefore these conditions are often incidentally found. It is important to consider a broad differential.This case is a 47 year old woman with history of mild intermittent asthma and chronic sinusitis who presented to the emergency department with four weeks of malaise, shortness of breath, cough, and two days of hemoptysis. On CT imaging she was found to have tracheal wall thickening, calcifications and projections along the trachea, as well as extensive right upper and middle lobe, and lingular ground glass opacities as seen in figure 1. With hindsight, similar tracheal abnormalities were noted on chest x-ray in 2019. During admission, ENT was consulted and she was noted to have saddle nose deformity. Nasal biopsy was performed and revealed evidence of chronic inflammation, but no other abnormalities. Extensive workup was performed including rheumatologic workup with mildly positive ANA, mildly elevated ESR and CRP, negative ANCAs, MPO, PR3, RF. Bronchoscopy was pursued and direct visualization of nodules were noted along the anterior and lateral aspects of the trachea, as well as narrowing of the right upper lobe segmental bronchi, also seen in figure 1. Bronchoalveolar lavage cultures revealed Citrobacter freundii complex, Group G beta hemolytic streptococcus, Providencia rettgeri, Enterococcus avium, Pseudomonas aeruginosa, Klebsiella pneumoniae, in addition to rhinovirus and COVID-19. Biopsy was not performed due to patient intolerance of the procedure and desaturation. Ultimately, the pulmonary infiltrates were felt to be a separate process from the tracheal nodules. She improved clinically, and was discharged on antibiotic therapy with a plan for close outpatient follow up.Tracheobronchopathia osteochondroplastica (TO) is a rare condition. Imaging is often the first clue, as symptoms come late in disease course. The differential diagnosis of imaging findings consistent with TO include relapsing polychondritis, granulomatosis with polyangiitis, amyloidosis, along with a host of others. Diagnosis of TO often requires multidisciplinary involvement to evaluate other etiologies,and ultimately bronchoscopy for direct visualization. (Table Presented).