1255. Dengue-Associated Hemophagocytic Lymphohistiocytosis in Puerto Rico
Session: Oral Abstract Session: Laboratory and Clinical Virology
Saturday, October 5, 2013: 11:45 AM
Room: The Moscone Center: 300
Background: Hemophagocytic lymphohistiocytosis (HLH) is a rare, potentially fatal disorder characterized by hyperinflammation due to uncontrolled proliferation of activated lymphocytes, resulting in prolonged fever, pancytopenia, jaundice, and hepatosplenomegaly. HLH can be familial or acquired, the latter being the result of malignancy or infection. Dengue-associated HLH (dengue-HLH) has been described in 26 case reports since 1966, but has not been previously recognized in Puerto Rico.

Methods: In December 2012, CDC Dengue Branch was notified of several dengue-HLH cases at two San Juan pediatric hospitals. An investigation was conducted to: 1) determine the incidence of HLH since 2008; and 2) determine the infecting agent(s) associated with HLH cases. Medical records were queried to identify patients with findings compatible with HLH.

Results: To date, 480 records have been reviewed and 18 patients identified that met accepted criteria for HLH. Sixteen (84%) HLH cases had diagnostic evidence of DENV infection by IgM ELISA (44%) or PCR (56%): dengue virus types -1and -4 were detected.  There was one fatal dengue-HLH case (case-fatality rate [CFR]: 5.5%). Dengue-HLH cases ranged in age from 0.2 -16 years, 50% were infants, and all resided in northern Puerto Rico. Among children aged 0–16 years, the annual incidence of dengue-HLH cases was 1.8 per 100,000 population. The median serum ferritin value was 22,524 µg/L (range: 754–522,000 µg/L) in dengue-HLH cases. Hemophagocytosis was evident in bone marrow aspirates of six of 11 (55%) dengue-HLH cases for which testing was performed. Median hospital stay was 26 days (range: 8–81 days). Only one hospital consistently used immunosuppressive therapy for suspected or confirmed HLH cases.

Conclusion: We are conducting a case-control study to identify risk factors for developing dengue-HLH and to determine why infants were predominantly affected. Some symptoms of HLH may also be seen in patients with severe dengue, potentially resulting in under-recognition. Physicians in dengue endemic areas should be made aware of HLH.

Esther M. Ellis, PhD, Division of Vector Borne Diseases- Dengue Branch, Centers for Disease Control and Prevention (CDC), Cataņo, PR


E. M. Ellis, None

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