CASE SERIES OF HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS IN ADULTS FROM A SINGLE CENTER

Abstract

SESSION TITLE: Wednesday Abstract Posters SESSION TYPE: Original Investigation Posters PRESENTED ON: 10/23/2019 09:45 AM - 10:45 AM PURPOSE: The purpose of this study was to review cases of secondary HLH treated at a tertiary care center and further characterize clinical factors that determine outcomes in patients requiring intensive care. METHODS: Potential cases of HLH were identified by searching hospital billing records for the ICD-9(288.4) & ICD-10(D76.1) codes from 2014 to 2018. Information was retrieved from the electronic medical record. Cases were screened against the HLH- 2004 criteria: fever, splenomegaly, cytopenias, hypertriglyceridemia/hypofibrinogenemia, hemophagocytosis in biopsy, low NK-cell activity, hyperferritinemia & elevated IL-2r (5 of 8 or molecular diagnosis). Relevant information including demographics, lab data, treatments & outcomes and were gathered and statistically analyzed RESULTS: 13 patients were identified via billing codes, 8 of which met diagnostic criteria above. The median age of diagnosis was 59 (range 21-77) with 53% being male(n=7). The majority (76%,n=10) identified as White, the remaining were African American(n=2) & Hispanic(n=1). The mean ferritin at diagnosis was 12321. The peak ferritin mean was 24043. Five had BM biopsy 2 of which were suggestive of HLH. Mortality rate was 61%(n=8). The most common signs and symptoms were fever (85% n=11) and splenomegaly(63% n=7). The most common lab abnormalities were hyperferritinemia(100% n=13), elevated liver enzymes(92% n=12), pancytopenia (92% n=12), elevated LDH(90% n=10), Hyperbilirubinemia(75%, n=9), low fibrinogen (53% n=7) and hypertriglyceridemia (50% n=6). Using unpaired t-test no significant difference was found between survivors and nonsurvivors in parameters tested, including initial ferritin (p=.34) & peak ferritin (p=.7). The survivors were younger (median 43 vs 63.5) with lower SOFA scores ( 7.6 vs 9.6) but these were not statistically significant against non survivors (p=0.1629 and p=0.4 ). Using Fisher exact test no significant difference was found between non survivors & survivors in characteristics tested including with High TG(>200) vs Low(<200)(p=1.00),Fever vs No Fever (p=0.4872), Splenomegaly vs No Splenomegaly(p=0.5758). Most patients were treated with dexamethasone(n=8) either alone or with etoposide (n=3). No therapy proved more efficacious in improving mortality. Underlying cause of HLH was recognized as malignancy in 4 patients and infection in 8. CONCLUSIONS: HLH is a disease with a highly variable presentation and a grim prognosis. A high index of suspicion should be used in patients with the constellation of splenomegaly, fevers & sepsis like syndrome; as well as the characteristic laboratory abnormalities, namely cytopenias, hyperferritinemia, hypertriglyceridemia, elevated LFT;s & elevated LDH. CLINICAL IMPLICATIONS: Secondary HLH is associated with high mortality, particularly when in older patients. Further research & collaborative registries are needed to help identify patients at higher risk of poorer outcomes. DISCLOSURES: No relevant relationships by Faris Al-Faris, source=Web Response No relevant relationships by Scott Beegle, source=Web Response No relevant relationships by Muhammad Salick, source=Web Response No relevant relationships by Ali Wazir, source=Web Response No relevant relationships by Robert Wilcott, source=Web Response