SESSION TITLE: Critical Care SESSION TYPE: Global Case Report Poster PRESENTED ON: Tuesday, October 31, 2017 at 01:30 PM - 02:30 PM INTRODUCTION: Hemophagocytic lymphohistiocytosis (HLH) due to prolonged activation of antigen presenting cell, causes a hyperinflammatory state resulting in cytopenias, organ destruction and death if not recognized early. HLH is an autosomal recessive disease in children and acquired in adults. Hematologic malignancy, followed by infection and rheumatologic disease, are the most common triggers in adults. High mortality is associated with malignancy and with delayed diagnosis. Clinical features include fever, hepatosplenomegaly, cytopenias, hyperferritinemia and hypofibrinogenemia. Additional findings include hyponatremia, elevated d dimer, LDH and CRP. We present a case of HLH secondary to a new diagnosis of B cell acute lymphoblastic leukemia. CASE PRESENTATION: 57 year old AA F with history of hypertension and diabetes with recent history of fatigue, shortness of breath, easy bruising and encephalopathy presented with asystolic arrest from the field with inferior wall ST elevation on return of circulation yet clean coronaries and a preserved ejection fraction. Labs, resulted during emergent cardiac catheterization, with a Hgb 2.2mg/dl, PLT 30K/uL, pH <6.8, Na133mE/L, D-Dimer >10mg/L FEU, Cr 1.4mg/dl, glucose 150mg/dl. LDH 4198U/L, PTT 45S, AST 839U/L, fibrinogen 127mg/dl, lactate 27 and INR 2.1IU. Oozing was present from her eyes, mouth, nose and vagina yet minimal bleeding on insertion of a dialysis catheter. The simultaneous presence of diffuse oozing and lack of bleeding on instrumentation, is clinically suggestive of disseminated intravascular coagulopathy (DIC) making reperfusion alone not the sole cause of this hyperinflammatory state. Empiric antibiotics, continuous renal replacement therapy and vasopressors were initiated. Despite massive transfusion of red cells, plasma, platelet and cryoprecipitate plus prothrombin complex concentration, Factor VIIa and IV vitamin K, clinical response to products was inappropriate and out of proportion to noted bleeding (no hemolysis). HLH due to malignancy was suspected given history and ferritin level of 97,000 ng/dl. Blood flow cytometry suggested B lymphoblastic leukemia (BALL). We initiated partial HLH treatment with dexamethasone 20 mg IV daily with noted improvement in hemodynamics and cessation of oozing. She was transferred to a tertiary care facility for acute B ALL treatment. Unfortunately, she succumbed to cardiac arrest prior to chemotherapy initiation. DISCUSSION: Pediatric diagnostic criteria have not been validated in adults2. Also, the criteria do not include many of the findings associated with HLH3. Presence of HLH associated mutations is diagnostic coupled with elevated soluble C25 (IL 2) receptor and ferritin levels as markers of inflammation are highly suggestive1. Unfortunately, genetic and sCD25 testing are not readily available, risking delay of treatment. Our patient met many of the modified diagnostic criteria of 20091 which coupled with her H Score of 225 (97% probability) and discovery of hematologic malignancy with profound inflammatory state leading to organ failure and refractory shock, coupled with hyperferrinitemia demanded treatment initiation for HLH. Steroids were used, etoposide was deferred given upcoming chemotherapy for B ALL. She presented late along the HLH spectrum with malignancy leading to her mortality. CONCLUSIONS: HLH adult literature is primarily retrospective, single center with small sample size further illuminating the importance of a high index of suspicion. This, coupled with meeting modified diagnostic criteria, typically associated findings and use of H score will strengthen clinical diagnosis allowing early treatment of the inflammatory storm and underlying trigger in adults. Reference #1: Filipovich, A. Hemophagocytic lymphohistiocytosis (HLH) and related disorders. Hematology. 2009.127-31 Reference #2: Rivera, S. et al. Reactive hemophagocytosis syndrome in adults: A retrospective analysis of 162 patients. Amer. J of Med. 2014;127:1118-1125 Reference #3: Hayden A, et al. Hemophagocytic syndromes including HLH in adults. A systematic scoping review. Blood Rev. 2016 DISCLOSURE: The following authors have nothing to disclose: Amanda Balzer-Costin, Doug Corwin, Heidi Abdelhady No Product/Research Disclosure Information