Critical Care: Student/Resident Case Report Poster - Critical Care V |

Hemophagocytic Lymphohistiocytosis: Three Case Reports and Review of the Literature in Adult Population 50 Years of Age and Above FREE TO VIEW

Reyna Altook, MD; Avneet Singh, MS; Wail Alamoudi, MBBS; Sultan Almaziad, MBBS; Zeinab Moussa, MD; Joan Duggan, MD; Fadi Safi, MD
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University of Toledo College of Medicine, Lancaster, OH

Copyright 2016, American College of Chest Physicians. All Rights Reserved.

Chest. 2016;150(4_S):457A. doi:10.1016/j.chest.2016.08.470
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SESSION TITLE: Student/Resident Case Report Poster - Critical Care V

SESSION TYPE: Student/Resident Case Report Poster

PRESENTED ON: Tuesday, October 25, 2016 at 01:30 PM - 02:30 PM

INTRODUCTION: Hemophagocytic lymphohistiocytosis (HLH) is a rare disease with an annual incidence of 1/50,000-1/150,000 and is fatal if not promptly treated. 1 Primary HLH occurs in infants with genetic predisposition, and presents with fever, splenomegaly, and pancytopenia. Acquired HLH is less well characterized and occurs in adults in the setting of severe inflammation triggered by infection or malignancy. Treatment of primary HLH is based on HLH-94 protocol, however little is known about the efficacy of such protocol in the adult population where early death was reported in those 50 years of age and above 2

CASE PRESENTATION: We report three patients over 50 years old admitted to the intensive care unit (ICU) with severe systemic inflammatory response syndrome (SIRS); including fever and pancytopenia; elevated liver enzymes, coagulopathy, and shock requiring vasopressors, without identifiable infectious source, who were ultimately diagnosed with acquired HLH (Figure1). We performed a literature review of HLH in adults over age 50 years in PubMed using the following key words (hemophagocytic lymphohistiocytosis) (Fever) (HLH in 50 years and older) and (adult onset HLH). We described the clinical characteristics, laboratory findings, treatment protocols, and clinical outcome of the disease.

DISCUSSION: A total of 71 cases (50 years and above) were identified as HLH. Mean age was 62 years, male to female ratio 3:2. Majority of patients (91%) have fever at presentation, and suffer from gastrointestinal signs and symptoms (72%). Most cases were triggered by infection (49%) followed by malignancy (27%). Mean serum ferritin level was 15756 ng/mL, and mean serum soluble interleukin 2 receptor (sCD25) was 11272 U/mL. Lactate dehydrogenase (LDH), and liver enzymes were reportedly high in most patients. Supportive treatment was done in 19 patients (26.7%). 12 patients (16.9%) received corticosteroids alone, while 45 patients (63.4%) received additional chemotherapeutic agent, mostly Etoposide and cyclophosphamide. IVIG was given for 19 patients (26.7%). Outcomes were 44 deaths (62%) and 27 survivals (38%).

CONCLUSIONS: HLH can mimic septic shock and acute liver failure in adult ICU patients, and should be considered in the differential diagnosis by early testing for serum Ferritin and soluble CD 25 levels. There is no standardize treatments in adults, reported therapy include combination of corticosteroids, Etoposide, Cyclophosphamide, and IVIG.

Reference #1: Filipovich A1, McClain K, Grom A. Histiocytic disorders: recent insights into pathophysiology and practical guidelines. 2010 Jan

Reference #2: Arca M, Fardet L, Galicier L, Rivière S, Marzac C, Aumont C, Lambotte O, Coppo P, Prognostic factors of early death in a cohort of 162 adult haemophagocytic syndrome: impact of triggering disease and early treatment with etoposide. Br J Haematol. 2015 Jan

DISCLOSURE: The following authors have nothing to disclose: Reyna Altook, Avneet Singh, Wail Alamoudi, Sultan Almaziad, Zeinab Moussa, Joan Duggan, Fadi Safi

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