title: Adult onset hemophagocytic lymphohistiocytosis prognosis is affected by underlying disease and coexisting viral infection: analysis of a single institution series of 35 patients.
abstract: Adult onset hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory syndrome, which can develop as a complication of many disorders. Early diagnosis is essential in order to avoid a fatal outcome. To confirm the diagnosis of acquired HLH made in a single institution series of adult patients with HLH-04 criteria, we applied the HScore and evaluated prognostic factors associated with clinical outcome. The median age of 35 patients was 54 (range 17-81), M/F ratio was 20/15. In 26/35 (74.3%) patients, an underlying haematological disease was present (2 Multicentric Castleman Disease, 10 B-cell Non-Hodgkin Lymphoma [NHL] and 14 T/NK-cell NHL); an autoimmune disorder was observed in four (11.4%) patients (one Still Disease, one undifferentiated connective tissue disease and two haemolytic anaemia); in five (14.3%), no underlying disease was identified. A concomitant infection by EBV was observed in 10 patients (28.6%), CMV in 8 (22.9%), HHV8 in 6 (17.1%) and HIV in 1 (2.9%). Hyperferritinemia, fever and splenomegaly were present in more than 90% of patients, whereas bone marrow hemophagocytosis in 51% of cases only. According to HScore, 34/35 patients had a >75% and 32/35 >93% probability of HLH. Four-year overall survival and HLH-free survival were 17.8% (CI 1.9-33.8) and 23.8% (CI 7.3-40.3), respectively. By multivariate analysis, presence of oedema and hyperbilirubinemia were predictors of death, whereas there was a statistically significant trend for viral infection as predictor of poor prognosis. B-NHL diagnosis was confirmed as associated to a better prognosis in comparison with T/NK lymphoma (4-year HFS 53.3% vs. 0%, p = 0.09) and similar to other aetiologies. Copyright © 2016 John Wiley & Sons, Ltd.
authors.affiliation: Hematology, Spedali Civili, Brescia, Italy.
authors.forename: Chiara
authors.initials: C
authors.lastname: Cattaneo
authors.affiliation: Hematology, Spedali Civili, Brescia, Italy.
authors.forename: Margherita
authors.initials: M
authors.lastname: Oberti
authors.affiliation: Unit of Blood Diseases and Stem Cell Transplantation, University of Brescia, Brescia, Italy.
authors.forename: Cristina
authors.initials: C
authors.lastname: Skert
authors.affiliation: Hematology, Spedali Civili, Brescia, Italy.
authors.forename: Angela
authors.initials: A
authors.lastname: Passi
authors.affiliation: Hematology, Spedali Civili, Brescia, Italy.
authors.forename: Mirko
authors.initials: M
authors.lastname: Farina
authors.affiliation: Hematology, Spedali Civili, Brescia, Italy.
authors.forename: Alessandro
authors.initials: A
authors.lastname: Re
authors.affiliation: Hematology, Spedali Civili, Brescia, Italy.
authors.forename: Paola
authors.initials: P
authors.lastname: Tozzi
authors.affiliation: Hematology, Spedali Civili, Brescia, Italy.
authors.forename: Erika
authors.initials: E
authors.lastname: Borlenghi
authors.affiliation: Hematology, Spedali Civili, Brescia, Italy.
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Rossi
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D020031:Epstein-Barr Virus Infections / Q000150:complications*; D005260:Female; D006801:Humans; D051359:Lymphohistiocytosis, Hemophagocytic / Q000401:mortality / Q000821:virology*; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D015996:Survival Rate; D055815:Young Adult
keywords: hemophagocytic lymphohistiocytosis; hemophagocytic syndrome; hemophagocytosis; immune activation; outcome of secondary hemophagocytic syndrome