Infectious Diseases Are Frequently Associated with Adult Hemophagocytic Lymphohistiocytosis in a Large Series of Cases
Flavia N Rosado, Annette S Kim, Elizabeth B Rinker, Natalie M Spradlin, Kaaren K Reichard. Mayo Clinic, Rochester, MN; Vanderbilt University Medical Center, Nashville, TN
Background: Hemophagocytic lymphohistiocytosis (HLH) is an inflammatory syndrome that can be divided into primary (familial, pediatric) and secondary (acquired, adult) types. Infectious diseases are commonly reported triggers in adults; however, data are limited to a few series. Immunosuppressive therapy improves survival in the pediatric cases.
Design: Confirmed cases of HLH in adults with documented bone marrow hemophagocytosis in a 10 year-period were retrospectively reviewed in two large academic centers. Pertinent clinical and laboratory findings were examined.
Results: Of 65 cases of bone marrow hemophagocytosis, 36 cases of HLH fulfilled at least 5 of the 8 diagnostic criteria (see table). The cases excluded lacked sufficient data to fulfill diagnostic criteria. Ages ranged from 19 to 85 (median 50), M:F ratio of 1.5:1. Half of the cases were attributed to infection (acute EBV infection followed by bacterial infections and histoplasmosis). In 36% the trigger was unclear. Fever was the most frequent symptom (96%). Elevated ferritin was present in all cases. The overall mortality rate was 62% (mean survival of 34 days) with 74% dying despite specific HLH therapy (see table).
Conclusions: This is the largest series of HLH cases in adults. Acute EBV and bacterial infections followed by histoplasmosis were the most frequent diseases identified in this series. Neoplasms and autoimmune diseases accounted for a minority of cases. The institution of specific immunosuppressive therapy did not change mortality rates, unlike what has been demonstrated in studies of the pediatric population.
Tuesday, March 5, 2013 2:20 PM
Proffered Papers: Section H2, Tuesday Afternoon