Hemophagocytic Lymphohistiocytosis in Intensive Care Unit

Hemophagocytic lymphohistiocytosis (HLH) is a critical condition that may lead to organ failure and early death. The aim of this retrospective observational study was to describe a cohort of HLH patients admitted to intensive care unit (ICU) and investigate the risk factors of early death. A positive HLH diagnosis was defined by an HScore 169. Univariate and multivariate analyses were carried out to investigate hospital and 28-day mortality risk factors. Between January 2002 and July 2014, 71 HLH cases were seen at our institution. The overall 28-day mortality (start at ICU admission) and hospital mortality were 38% and 68%, respectively. The factors associated with increased 28-day mortality were the sequential organ failure assessment score at ICU admission (P< .001) and advance in age (P 1⁄4 0.03). The factors associated with increased hospital mortality were a high sequential organ failure assessment score at ICU admission (P< 0.01), advance in age (P1⁄4 0.04), and the presence of lymphoma-related HLH or HLH of unknown origin (P< 0.01). Organ failure overtops the classical early-death risk factors in adult ICU-admitted HLH patients. This failure and the subsequent early death may be prevented by timely specific cytotoxic therapies and the control of the underlying disease. (Medicine 94(51):e2318) roussolle, MD, Yva D, D, and Pascal Sève, MD, PhD HIV = human immunodeficiency virus, HLH = hemophagocytic lymphohistiocytosis, HScore = reactive hemophagocytic syndrome diagnostic score, ICD-10 = International Classification of Diseases Tenth revision, ICU = intensive care unit, NK cells = natural killer cells, SGOT = serum glutamic oxaloacetic transaminase, SOFA = sequential organ failure assessment. INTRODUCTION H emophagocytic lymphohistiocytosis (HLH) is a life-threatening condition characterized by a hyperinflammatory state mediated by impaired natural-killer (NK) and cytotoxicT-cell functions. The main clinical features of HLH include high fever, hepatosplenomegaly, bicytopenia, and the presence of activated macrophages in hematopoietic sites; these constitute the classical HLH criteria. Among the 2 hemophagocytic syndrome subsets, primary/genetic or secondary/reactive, the latter is the most frequently seen in adults. This condition may be triggered by various underlying conditions such as infection (especially by Epstein-Barr virus (EBV), cytomegalovirus (CMV), or human immunodeficiency virus (HIV)), lymphoid malignancy (Bor T-cell lymphoma), connective tissue diseases, or some drugs. HLH criteria were established by the Histiocyte Society in 2004. More recently, Fardet et al proposed an HScore, a diagnostic tool based on 9 criteria: clinical: known underlying immunosuppression, high temperature, organomegaly; biological: cytopenia, high levels of triglyceride, ferritin, and serum glutamic oxaloacetic transaminase (SGOT), and low level of fibrinogen; and cytological: hemophagocytosis features on bone marrow aspirates. In this study, expert agreement was considered as the gold standard for the diagnosis of HLH. This HScore was the first validated in adult reactive HLH but not yet in familial lymphohistiocytosis. Because of HLH severity, intensivists are increasingly asked to take care of HLH patients. Indeed, in the absence of specific treatment, the expected outcomes of HLH are multiple organ failure and, ultimately, death; in a recent literature review, Ramos-Casals et al reported a 41% mortality rate in 1109 patients. Whereas several studies have investigated the prognostic factors of HLH, little is known about the prognosis of critically ill HLH patients. except for a high hospital mortality rate (52% in 56 patients). In particular, the HLH mortality-associated risk factors in ICU are still poorly known due to the small sizes of the available series. The present study describes a relatively large retrospective admitted to several polyvalent ICUs of a spitals and attempts to identify the early n HLH. www.md-journal.com | 1