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• W4387248610 abstract "SESSION TITLE: Critical Care Case Report Posters 69 SESSION TYPE: Case Report Posters PRESENTED ON: 10/11/2023 12:00 pm - 12:45 pm INTRODUCTION: Deferiprone is an iron chelation therapy that is used for iron overload in patients receiving frequent transfusions. Deferiprone-induced agranulocytosis is a rare cause of neutropenic fever, the pathogenesis of which remains unidentified, occurring in 1-2% of patients on therapy [1]. Here we present a case elucidating this life-threatening complication of iron chelation therapy. CASE PRESENTATION: A 58 year old man with a history of transfusion dependent beta thalassemia intermedia, presented to the emergency department for evaluation of several days of progressive weakness, diarrhea and confusion. He was found to be febrile to a peak temperature of 40C, tachycardic as high as HR 116, and only oriented to self. Significant pancytopenia was evident on initial laboratory analysis: leukopenia to 0.47 x103/uL, hemoglobin 7.3 g/dL, thrombocytopenia to 65 x103/uL and agranulocytosis with 0.00 x103/uL neutrophils. Procalcitonin notably elevated above 100 ng/mL.The possibility of neutropenic fever with underlying sepsis was considered and a critical care consult requested. Shortly after, the patient developed fluid resistant hypotension as low as 72/50. Vasopressors were initiated and the patient was transferred to the intensive care unit.The patient underwent extensive infectious workup and empiric antibiotics administered - cefepime, vancomycin, ampicillin, and micafungin. His mental status improved as blood pressure normalized, and his initial presentation of diarrhea resolved. Extensive septic workup remained unrevealing. On review of prior records, patient's thalassemia was complicated by secondary hemochromatosis with extramedullary hematopoietic masses, splenomegaly, on chelation therapy with deferoxamine and recently started on deferiprone one year ago. Chelation-induced neutropenia was suspected. Chelation therapy was held, and patient was started on tbo-filgrastim with gradual improvement. DISCUSSION: Neutropenia is known to cause isolated fevers. However, as our patient was in shock, he was treated with broad spectrum antibiotic coverage. In addition to choices based on the local antibiogram, coverage of siderophilic organisms such as Listeria monocytogenes, Salmonella enterica, Vibrio vulnificus and Yersinia enterocolitica should be considered for patients with hemochromatosis [2]. Upon improvement of neutropenia, the patient should also be observed for immune reconstitution syndrome, which may present as a dysregulated inflammatory response to a pre-existing infection with multiorgan dysfunction [3]. CONCLUSIONS: We highlight a case of chelation-induced neutropenia in a patient with septic shock. It is imperative to understand the risk of agranulocytosis in patients undergoing chelation therapy as well as the unique empiric antibiotic options available. Vigilance for any development of immune reconstitution syndrome as neutropenia improves is key for positive outcomes. REFERENCE #1: Tricta F, Uetrecht J, Galanello R, et al. Deferiprone-induced agranulocytosis: 20 years of clinical observations. Am J Hematol. 2016;91(10):1026-1031. REFERENCE #2: Ganz T, Nemeth E. Iron homeostasis in host defence and inflammation. Nat Rev Immunol. 2015;15(8):500-510. REFERENCE #3: Sun HY, Singh N. Immune reconstitution inflammatory syndrome in non-HIV immunocompromised patients. Curr Opin Infect Dis. 2009;22(4):394-402. DISCLOSURES: No disclosure on file for Olumayowa Abe No relevant relationships by Anirban Basu No relevant relationships by JohnPaul Kwak No relevant relationships by Mohamed Salih Makawi No relevant relationships by Angelina Voronina" @default.
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• W4387248610 date "2023-10-01" @default.
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• W4387248610 title "IRONMAN IS EMPTY INSIDE: WHEN CHELATION THERAPY GETS GREEDY" @default.
• W4387248610 doi "https://doi.org/10.1016/j.chest.2023.07.1378" @default.
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