PULMONARY MUCORMYCOSIS IN A PATIENT WITH SICKLE CELL CRISIS AND ACUTE CHEST SYNDROME

SESSION TITLE: Chest Infections Case Report Posters 21 SESSION TYPE: Case Report Posters PRESENTED ON: 10/10/2023 09:40 am - 10:25 am INTRODUCTION: Pulmonary mucormycosis is a devastating diagnosis, carrying an abysmal prognosis, and is mainly described in patients with immunodeficiencies—few cases of mucormycosis complicating sickle cell disease are reported. We present a young woman with sickle cell crisis, hypoxemia, and cavitary lesions, with a positive Mucor polymerase chain reaction test on bronchoalveolar lavage. CASE PRESENTATION: A 22-year-old female with sickle cell disease presented to an outlying hospital with acute hypoxic respiratory failure, raising concerns about acute chest syndrome and vaso-oclusive crisis. She was initially receiving intravenous antibiotics and red blood cell transfusions. Due to her worsening condition, she was transferred to our tertiary care center and subsequently intubated. The patient had a history of vaping and marijuana use and had an outpatient medication regimen, including opioids and hydroxyurea. She was synchronous with the ventilator, tachycardic, and had rales bilaterally. Extremities had minor pitting edema bilaterally. Her laboratory studies were significant for BUN 19 mg/dL, Creatinine of 0.6 mg/dL, total bilirubin of 6.1 mg/dL, and indirect bilirubin of 3.2 mg/dL, lactic acid of 0.6 mMol/L. Haptoglobin was <30 mg/dL, hemoglobin S was 13%, and hemoglobin was 9.1 gm/dL. SARS-CoV-2 RNA PCR was not detected. A computed tomography angiogram revealed diffuse ground glass opacities and areas of interlobular septal thickening. Multifocal irregular consolidative opacities of varying size in bilateral lungs, some of which demonstrated internal cavitation. Consolidative opacities along the bronchovascular bundle of the right lower lobe. She underwent red cell exchange and was started on broad-spectrum antibiotics. Despite three days of broad-spectrum antibiotics, she continued to have persistent fevers above 101 F and shock. Flexible bronchoscopy demonstrated bilateral friable mucosa and erythema. BAL cell count had 25% polymorphonuclear cells, 63% Macrophages. Polymerase chain reaction testing on the bronchoalveolar lavage fluid was positive for Mucor. All other fungal assays were negative, including Aspergillus PCR, 1-3-Beta-D-Glucan, and Histoplasma antigen. She was started on Liposomal amphotericin B 5mg/kg/day on day 15 of her admission. Her condition progressed to multiorgan failure despite antifungal therapy, and she passed away on day 28 of her hospital stay. DISCUSSION: The main risk factors for mucormycosis are hematological malignancies, organ transplantation, diabetes mellitus, and, most recently, COVID-19. Sickle cell disease as a risk factor for mucormycosis acquisition has not been extensively described. Here we present the case of a 22-year-old woman with acute chest syndrome, ultimately diagnosed with possible pulmonary mucormycosis. We speculate that our patient's risk factor for the acquisition of mucor was marijuana smoking, which has been reported as a risk factor in immunocompromised adults with Aspergillus, Fusarium, and Mucor pulmonary infections. Microbiology diagnostic tools have limited sensitivity. Molecular studies aid in the early diagnosis and treatment of fungal infections. CONCLUSIONS: This case highlights the importance of molecular assays for early diagnosis—delayed therapy results in a 2-fold increase in mortality. In patients with pulmonary cavitations and acute chest syndrome secondary to sickle cell disease, mucormycosis should be included in the differential diagnosis. REFERENCE #1: Danion F, Coste A, Le Hyaric C, Melenotte C, Lamoth F, Calandra T, Garcia-Hermoso D, Aimanianda V, Lanternier F, Lortholary O. What Is New in Pulmonary Mucormycosis? J Fungi (Basel). 2023 Feb 28;9(3):307. doi: 10.3390/jof9030307. PMID: 36983475. REFERENCE #2: Walsh TJ, Gamaletsou MN, McGinnis MR, Hayden RT, Kontoyiannis DP. Early clinical and laboratory diagnosis of invasive pulmonary, extrapulmonary, and disseminated mucormycosis (zygomycosis). Clin Infect Dis. 2012 Feb;54 Suppl 1:S55-60. doi: 10.1093/cid/cir868. PMID: 22247446. REFERENCE #3: Stone T, Henkle J, Prakash V. Pulmonary mucormycosis associated with medical marijuana use. Respir Med Case Rep. 2019 Jan 9;26:176-179. doi: 10.1016/j.rmcr.2019.01.008. PMID: 30671341; PMCID: PMC6330507 DISCLOSURES: Stock holder relationship with Amgen Pharmaceuticals Please note: $5001 - $20000 by Joe Chiles, value=25 shares of stock No relevant relationships by Luis Lantigua Tatem No relevant relationships by Christopher Richardson No relevant relationships by Nhi Ngoc Vu-Ticar