Adams, Alberto Quaglia, Charalambos G. Antoniades Background: HLH is frequently fatal (overall mortality
>50%) and is often underdiagnosed. It involves a final common pathway of hypercytokinemia. A timely diagnosis is imperative to facilitate immunosuppressive therapy and decrease mortality. HLH presenting as severe acute hepatitis or ACLF is extremely rare and is not well recognized. We present our experience of HLH in patients presenting with severe acute hepatic insult/ liver failure. Patients and Methods: Retrospective analysis of admitted patients Cilomilast concentration with systemic inflammatory response fulfilling diagnostic HLH criteria (> 5/8).[Henter et al, 2004] Results Seventeen patients [M: F -14:3; Adults: Child- 14:3; median age- 26 years (range-1 m to 66 yrs] were diagnosed with acquired HLH at our hospital from
year 2010 to 2012. Twelve (70.6%) patients presented clinically as ACLF (n=7) and severe acute hepatitis ( n=5) at admission. Viral associated HLH (8 patients; 47.1%) was most common (HAV-3, HEV-2, EBV-1, Dengue-1, Parvovirus-1). Although, etiology remained undiag-nosed in 7(41.2%) patients, rare presentations included lym-phomatous infiltration of liver (1) & visceral leishmaniasis (1). While fever [16 patients (94.2%); median duration- 30days (4-90 days)] and jaundice [14 patients; mean bilirubin-20.34 ± 8.6mg/dl] were most common presentations, clinical signs such as presence of enlarged liver (76.4%), spleen (76.4%) and ascites (58.8%) were most frequent. Five ACP-196 concentration patients also had AKI (serum creatinine >1.5 mg/dl) at admission. Important biochemical parameters included hyperferritinemia [all patients; mean- 16,064.7 ±7,652 ng/ml], hypertriglyceridemia (10 patients; mean-355 ± 145 mg/dl), raised LDH(mean- 2,953 ± 726 IU/L) and low fibrinogen (12 patients; mean-146.1 ± 32.4 mcg/L) levels. The mean plasma haemoglobin was 8.08 ± 1.33 g/L and total leucocyte count was 2.1
x 103 /cu mm. Bone marrow aspiration was done in 12 patients; 11 of which showed the presence of hemophagocytosed histiocytes. Ten (58.8%) patients had in-hospital mortality and the main cause was eventual sepsis and multiorgan failure. 12 patients (70.6%) received specific immunosuppressive therapy (steroids-4, IV immunoglobulin -4, plasmaphersis-3, cyclosporine-1) but these therapies made no difference in clinical outcome compared to those who did selleck kinase inhibitor not receive these therapies (in-hospital mortality- 66.67% vs. 62.25%, respectively). Conclusions: HLH may masquerade as acute hepatic insult, in patients presenting with severe and rapidly progressive liver failure. It is important to suspect and recognise this generally fatal entity early enough in patients with unexplained acute hepatitis or ACLF, especially in presence of severe anemia. Disclosures: The following people have nothing to disclose: Ankur Jindal, Ashok Choudhary, Shiv K. Sarin Background/Aim The prognostic assessment of cirrhotic patients in the ICU provides short-term and controversial results.