SESSION TITLE: Fellows Pulmonary Manifestations of Systemic Disease Posters
SESSION TYPE: Fellow Case Report Posters
PRESENTED ON: October 18-21, 2020
INTRODUCTION: Infection and trauma are the most common causes of acute respiratory distress syndrome (ARDS). However, ARDS has a lengthy differential diagnosis and rare etiologies must be considered when more common causes are excluded or with atypical clinical presentations.
CASE PRESENTATION: A healthy 18 y/o male presented to the hospital with a five day history of cough, hemoptysis and fever. In the ER he was found to have hypoxic respiratory failure. He was placed on the regular medical floor on supplemental oxygen via nasal canula. Initial chest x-ray showed bilateral upper lobe interstitial infiltrates. In the emergency room he was tested for COVID-19 and other common respiratory viruses. His COVID-19 was negative but his screen came back positive for adenovirus. Through the first day of his admission his oxygen requirements continued to increase. He was intubated and diagnosed with severe acute respiratory distress syndrome (ARDS). He was started on broad spectrum antibiotics. Despite ARDSnet protocol and supportive care his oxygenation did not improve. His ferritin levels increased to greater than 20,000 ng/dl and his d-dimer nadired at 17.2 mg/L. Because of these abnormalities and three total negative COVID-19 tests, a hematology consult was placed. Hematology diagnosed patient with secondary HLH from adenovirus. He was started on etoposide and dexamethasone per HLH-94 guidelines. His oxygenation worsened and he was placed on veno-venous extra-corporeal membrane oxygenation (ECMO). Despite antivirals, chemotherapy and ECMO the patient ultimately succumbed to his illness on day 20 of his admission.
DISCUSSION: Inherited and acquired HLH is a common known disease process in children, even though it is rarely seen. In adults HLH is often sought but seldom actually found. Adenovirus induced HLH is a rare disease in children, and unheard of in adults. Treatment of HLH is based off of the HLH-94 protocol, developed in 1995 by the Hemophagocytic Lymphohistiocytosis Study Group. New recommendations were made in 2004. The intent of these guidelines was to add newer immunotherapy with well established chemotherapeutic regiments. To date no clear guidelines for the treatment of HLH in adults has been set forth, therefore the pediatric treatment guidelines are often used for adult treatment as well. Novel treatment approaches have been reported for HLH induced by Epstein Barr Virus in adults, but none exist for adenovirus induced HLH. ARDS is not a common manifestation of HLH. The diagnosis is difficult to make because of poor recognition, therefore its prevalence in adults is uncertain. Unfortunately, as in this case, the disease is almost universally fatal. With the current healthcare environment being inundated with COVID-19, careful consideration and investigation was needed to arrive at the correct diagnosis.
CONCLUSIONS: ARDS is an uncommon presentation of an uncommon disease, secondary HLH.
Reference #1: Hayden A, Park S, Giustini D, Lee AY, Chen LY. Hemophagocytic syndromes (HPSs) including hemophagocytic lymphohistiocytosis (HLH) in adults: a systematic scoping review. Blood Rev (2016) 30:411–20. doi:10.1016/j.blre.2016.05.001
Reference #2: Trottestam H, Horne A, Aricò M, et al. Histiocyte SocietyChemoimmunotherapy for hemophagocytic lymphohistiocytosis: long-term results of the HLH-94 treatment protocol., Blood, 2011, vol. 118 17(pg. 4577-4584)
DISCLOSURES: No relevant relationships by Anna Plemmons, source=Web Response
No relevant relationships by William Pruett, source=Web Response
No relevant relationships by Michael Sanley, source=Web Response