ADULT ONSET HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS SECONDARY TO MULTI-ORGANISM SEPSIS IN A NEWLY DIAGNOSED HIV/AIDS PATIENT

Abstract

SESSION TITLE: Wednesday Medical Student/Resident Case Report Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: 10/23/2019 09:45 AM - 10:45 AM INTRODUCTION: Hemophagocytic lymphohistiocytosis (HLH) in the setting of sepsis and shock portends an overall poor prognosis. This is especially true in the setting of underlying severe immunodeficiency as is seen with HIV/AIDS. We herein present a case of exacerbated multi organism septic shock in the setting of HLH and a newly diagnosed HIV/AIDS patient. CASE PRESENTATION: A 36 year old previously healthy female presented with complaints of fever, chills, productive cough and malaise over the preceding 10 days. Labs on presentation were significant for pancytopenia, elevated ferritin (>40000 ng/mL) and hypertriglyceridemia. A CT chest (fig. 1) revealed multiple peripheral lung lesions. Further testing revealed new diagnosis of HIV with corresponding CD4 count of 11 cells/μL. Microbiologic evaluation revealed blood cultures positive for escherichia coli, candida albicans, histoplasma capsulatum (fig. 2). Patient was further found to be viremic with EBV and CMV titers of 34000 copies/mL and 18900 copies/mL respectively; stool cultures were positive for cryptosporidium. BAL was performed and further demonstrated presence intracellular budding yeast in both neutrophils and macrophages. Microbiology further identified aspergillus galactomannan, blastomyces dermatitidis, histoplasma capsulatum and pneumocystis carinii; Urine positive for budding yeast. Patient was started on vancomycin, piperacillin/tazobactam, azithromycin, and amphotericin B. Despite treatment, fevers persisted and respiratory status declined rapidly requiring mechanical ventilation. Despite aggressive treatment, patient entered septic shock. Given acuity of patient illness, she remained ineligible for any hematologic intervention. Maximal therapy was continued until hemodynamic compromise became unavoidable. DISCUSSION: HLH is a well documented hematologic emergency that requires robust intervention. HLH most typically results from a precipitating insult such as a viral infection. What results is uncontrolled lymphocyte and macrophage activation with consequent BM suppression (subscript 1).Here, EBV, CMV, and HIV titers were all elevated in addition to multiple bacterial, fungal and protozoal pathogens. Regarding diagnosis of HLH, this patient exhibited 5 of 8 criteria sufficient for consideration of the diagnosis.(subscript 2) We believe that her rapid decompensation and multi-organism sepsis was acutely exacerbated by the development of HLH. CONCLUSIONS: In spite of recently documented improvements in clinical outcomes for HIV positive patients receiving treatment in the ICU (supscript 3), in the case of HLH in the setting of multi-organism sepsis with bacterial, viral and fungal etiologies, early diagnosis and precise therapy are key in successful treatment of this condition. Our case highlights the difficulty of treating HLH in the setting of advanced HIV. Reference #1: Karakike, Eleni, and Evangelos J. Giamarellos-Bourboulis. “Macrophage Activation-Like Syndrome: A Distinct Entity Leading to Early Death in Sepsis.” Frontiers in Immunology, vol. 10, 2019, https://doi.org/10.3389/fimmu.2019.00055. Reference #2: Meena, Nikhil K., et al. “The Performance of Diagnostic Criteria for Hemophagocytic Lymphohistiocytosis in Critically Ill Patients.” Journal of Intensive Care Medicine, 2019, p. 088506661983713., https://doi.org/10.1177/0885066619837139. Reference #3: Silva, João Manoel, and Sigrid De Sousa Dos Santos. “Sepsis in AIDS Patients: Clinical, Etiological and Inflammatory Characteristics.” Journal of the International AIDS Society, vol. 16, no. 1, 2013, p. 17344., https://doi.org/10.7448/ias.16.1.17344. DISCLOSURES: No relevant relationships by Soumojit Ghosh, source=Web Response no disclosure on file for Scott Maughan; No relevant relationships by David picker, source=Web Response