ORIGINAL ARTICLE   

Gazzetta Medica Italiana - Archivio per le Scienze Mediche 2019 May;178(5):327-34

DOI: 10.23736/S0393-3660.18.03798-1

Copyright © 2018 EDIZIONI MINERVA MEDICA

language: English

Hemophagocytic syndrome in adults. Experience of a tertiary center

Sara COELHO ^{1, 2} ✉, Pedro MARQUES ¹, Patrícia LOURENÇO ¹, Paulo BETTENCOURT ^{3, 4}

¹ Department of Internal Medicine, Hospital of São João, Porto, Portugal; ² Department of Medical Oncology, Portuguese Institute of Oncology of Porto, Porto, Portugal; ³ Department of Medicine, University of Porto, Porto, Portugal; ⁴ Hospital CUF Porto, Porto, Portugal

BACKGROUND: Hemophagocytic lymphohistiocytosis (HLH) is a rare potentially life-threatening immune-mediated disorder extensively unrecognized in adults. We aimed to describe the frequency of HLH in an adult population, the conditions associated with it and its prognosis.
METHODS: We retrospectively studied adults diagnosed with HLH according to HLH-2004 guidelines. We reviewed hospital records between January 2000 and April 2015 and collected clinical, laboratory and pathologic data. Variables were evaluated using descriptive statistics and univariate analysis.
RESULTS: We identified 20 HLH cases (2.46 cases/100,000 hospital admissions per year). Half of the cases were recognized by clinicians. Clinical and laboratory characteristics were: fever (100%), splenomegaly (95%), cytopenia of two or more lineages (100%), hyperferritinemia (100%), increased lactate dehydrogenase (95%), hypofibrinogenemia (70%), hypertriglyceridemia (50%) and bone marrow hemophagocytosis (95%). Malignancies (45%) and infections (35%) were the most frequently associated triggers. Viral reactivation was common (52.6%), being predominant in infectious (66.7%) and auto-inflammatory (75%) triggers. Global mortality was 55%, with 54.5% of fatality-cases related to neoplastic triggers. Viral reactivation was frequent in fatal HLH cases (60%). HLH-specific therapy was given to 35% of patients, 57.1% surviving. Patients with no specific treatment had 38.5% survival.
CONCLUSIONS: Malignancy and viral reactivation were more frequent triggers in fatal cases, suggesting an association between mortality and these factors. Superimposition of HLH with other diseases, and broad clinical spectrum and triggers can delay diagnosis or even lead to underrecognition and undertreatment of HLH in adults. Our results suggest the need to increase awareness of this condition in order to use treatments that can improve outcome.

KEY WORDS: Lymphohistiocytosis, hemophagocytic - Epstein-Barr virus infections - Cytomegalovirus infections - Immunosuppression