A CASE OF FATAL SEPTIC SHOCK IN SECONDARY HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS (HLH) ASSOCIATED WITH BACTERIAL INFECTION IN THE ICU SETTING

Abstract

SESSION TITLE: Medical Student/Resident Critical Care Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: HLH is a rare systemic inflammatory disease with a high rate of mortality. Early diagnosis and urgent intervention are therefore crucial for survival. Primary HLH is mostly seen in children and is caused by an underlying genetic mutation. Secondary HLH is a rare clinical condition, and its underlying pathophysiology isn’t well studied, but common triggers include underlying infections, malignancies, and autoimmune diseases. We present a case of an 80 yo F presenting with altered mental status (AMS) and fever progressing to septic shock. CASE PRESENTATION: 80 yo F from Bangladesh with PMH of HTN, Asthma, Afib, CVA admitted with worsening AMS for 3 months. Urine culture grew E.coli. Chest X-ray revealed bilateral pneumonia. She was started on antibiotics. Labs on presentation were notable for pancytopenia, requiring transfusion of blood products, ferritin >9900, Total Bilirubin/Direct/ALKP/ALT/AST - 20.4/16.3/857/808/3485. HIV, Hep A, B, C negative with unremarkable right upper quadrant ultrasound. CT chest showing mediastinal lymphadenopathy with the largest subcarinal node of 2.5 cm. Despite aggressive treatment, the patient developed septic shock. Her hemodynamic status deteriorated requiring mechanical ventilation and vasopressors. Bone marrow biopsy (BMP) was considered to rule out underlying hematologic disorder given mediastinal lymphadenopathy and bicytopenia. The patient expired before the biopsy results were reported. BMP confirmed the diagnosis of HLH showing histiocytes with hemophagocytosis. DISCUSSION: Secondary HLH can be triggered by infections, autoimmune diseases and malignancies, with the majority of cases occurring in ICU going underdiagnosed and carrying a poor prognosis with a high mortality rate. Manifestation varies between individual cases and It can present with a combination of various symptoms and signs. Secondary HLH is difficult to identify as it can mimic septic shock with progressive multiorgan failure. HLH 2004 diagnostic criteria require 5/8 findings (Table 1). Modified criteria are useful as it’s common for a patient with HLH to exhibit only 3-4/8. Retrospective studies have identified infections as common precipitating factors, with one study showing bacterial organisms to be the predominant trigger among infectious agents, and another one showing viral infection as a more common provoking factor. The mainstay of treatment involves immuno/myelosuppressive agents. CONCLUSIONS: Secondary HLH is a multisystem disorder caused by uncontrolled immune activation. Although big prospective studies for data analysis of diagnostic modalities and prognostic factors are lacking, a high index of suspicion for HLH should be maintained for adults presenting with the above-mentioned manifestations. Further understanding and refined diagnostic criteria are important for early diagnosis and implementing disease-modifying therapies for this life-threatening syndrome. Reference #1: Raschke RA1, Garcia-Orr R2. Hemophagocytic lymphohistiocytosis: a potentially underrecognized association with systemic inflammatory response syndrome, severe sepsis, and septic shock in adults. Chest. 2011 Oct;140(4):933-938. Reference #2: Jae-Ho Yoon, Sung-Soo Park, Young-Woo Jeon, Sung-Eun Lee, Byung-Sik Cho, Ki-Seong Eom, Yoo-Jin Kim, Hee-Je Kim, Seok Lee, Chang-Ki Min, Seok-Goo Cho, and Jong Wook Lee. Treatment outcomes and prognostic factors in adult patients with secondary hemophagocytic lymphohistiocytosis not associated with malignancy. Chest. 2011 Oct;140(4):933-938. Reference #3: Sameer A Parikh, Prashant Kapoor, Louis Letendre, Shaji Kumar, Alexandra P Wolanskyj. Prognostic Factors and Outcomes of Adults With Hemophagocytic Lymphohistiocytosis. Mayo Clinic Proceedings 2014, 89 (4): 484-92 DISCLOSURES: No relevant relationships by Mariam Agladze, source=Web Response No relevant relationships by Theo Trandafirescu, source=Web Response