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• W2894813051 abstract "SESSION TITLE: Critical Care 1 SESSION TYPE: Fellow Case Report Posters PRESENTED ON: 10/09/2018 01:15 PM - 02:15 PM INTRODUCTION: Hemophagocytic lymphohistiocytosis (HLH) is a potentially fatal syndrome of uncontrolled hyperinflammation (1). It is often difficult to distinguish between HLH and sepsis. HLH presents a unique challenge to physicians in that the underlying etiology must be determined in order to administer therapy. CASE PRESENTATION: We present a 48-year-old male with a history of hypertension who presented with two months of recurrent fevers. He was admitted for work up of fever of unknown origin. Physical exam was unremarkable except for splenomegaly. He was hospitalized for three weeks without a definitive diagnosis despite an extensive infectious, rheumatologic, and hematologic work up. He was discharged home with a plan for outpatient follow up. Two weeks after discharge he returned with persistent fevers, hemodynamic instability, and multisystem organ dysfunction. Lab values were notable for pancytopenia, hyperferritinemia, and hypertriglyceridemia. Given his labs, fever, and splenomegaly, there was a strong suspicion for HLH. The peripheral blood showed rare large lymphoma-like cells, some with granules. A bone marrow biopsy was performed which confirmed the presence of hemophagocytic syndrome and demonstrated an interstitial atypical large lymphoid infiltrate with natural killer (NK) cell immunophenotype by flow cytometry (positive for CD2, CD7, dim CD8, CD16, CD56) and by immunohistochemistry (positive with CD2, CD7, cytoplasmic CD3 and EBV-encoded small RNA (EBER) by in-situ hybridization) diagnostic of aggressive NK-cell leukemia. Two days following the diagnosis of leukemia the patient passed away. DISCUSSION: HLH is a syndrome that is characterized by over-stimulated cytotoxic cells (1). Clinically, patients present with fever, splenomegaly, hemophagocytosis within the bone marrow, and specific lab abnormalities. The treatment for HLH includes the use of dexamethasone, etoposide, and cyclosporine A (1). NK-cell leukemia, known to be associated with HLH, is a rare disease entity that carries a poor prognosis. It is more prevalent among Asians, with no sex predilection, and occurs at a median age of 40 years. Classically, leukemic cells present with evidence of EBV infection (2). The median survival of patients evaluated within a case series was 58-days (2). The disease is frequently complicated by coagulopathy, hemophagocytic syndrome, or multiorgan failure (3). It is unclear whether identification of HLH or the diagnosis of NK-cell leukemia in our patient earlier on would have ultimately changed his clinical course. However, establishing these diagnoses earlier would have likely changed his management and greatly aided in prognostication. CONCLUSIONS: HLH is a rare syndrome that shares overlapping features with sepsis. When a patient with suspected sepsis does not respond to appropriate sepsis-directed therapy, one should be prompted to consider the possibility of HLH and an underlying cause. Reference #1: Machowicz R, Janka G, Wiktor-Jedrzejczak W. Similar but not the same: Differential diagnosis of HLH and sepsis. Crit Rev Oncol Hematol. 2017;114:1-12. Reference #2: Suzuki R, Suzumiya J, Nakamura S, et al. Aggressive natural killer-cell leukemia revisited: large granular lymphocyte leukemia of cytotoxic NK cells. Leukemia. 2004;18(4):763-770. Reference #3: Chan JKC, Jaffe ES, Ko, Y-H. Aggressive NK-cell leukemia. WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues, IARC. 2017: 753-754. DISCLOSURES: No relevant relationships by Judith Brody, source=Web Response No relevant relationships by Craig Fryman, source=Web Response No relevant relationships by Adey Tsegaye, source=Web Response" @default.
• W2894813051 created "2018-10-12" @default.
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• W2894813051 date "2018-10-01" @default.
• W2894813051 modified "2023-10-13" @default.
• W2894813051 title "A RARE CASE OF HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS AND NATURAL KILLER-CELL LEUKEMIA" @default.
• W2894813051 doi "https://doi.org/10.1016/j.chest.2018.08.264" @default.
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