Papers of the Week

Hemophagocytic lymphohistiocytosis (HLH) is an uncommon fatal disease of otherwise normal but hyperactive lymphocytes and histocytes. HLH could be primary (hereditary) or secondary (acquired). Fever, hepatosplenomegaly, lymphadenopathy, and neurologic dysfunction are among the common symptoms of HLH. The diagnosis of HLH is based on clinical and biochemical findings. We report here a case of a patient infected with the dengue virus who developed HLH during hospitalization. A 63-year-old female known case of asthma on inhalers, chronic hepatitis B virus, gastritis on proton pump inhibitors, and hemoglobin H disease presented to the emergency department (ED) with a history of high-grade fever (highest recorded temperature 40° C/ 104° F), which was relieved partially by antipyretics, generalized fatigability, body aches, headache and mosquito bites for four days. The physical examination was significant for hepatomegaly of 4 cm below the right costal margin. Investigations revealed pancytopenia with elevated ferritin levels (> 40000 µg/L). Viral serology was positive for dengue NS1 antigen. After hematology consultation, a bone marrow biopsy was done, which showed trilineage hematopoiesis with increased histiocytes and occasional hemophagocytosis. Given that the patient was clinically stable and there was a clear triggering condition, we opted for supportive measures rather than HLH-specific therapy. The patient was given 2 units packed red blood cells for anemia. On the following days, the patient has no recurrence of fever, with marked improvement in the biochemical profile including ferritin level (1165 µg/L). HLH is a deleterious disease with a high fatality rate, which requires the clinician to have a low threshold for suspicion in the differentials of children and adults with symptoms of persistent fever, hepatosplenomegaly, and cytopenia. Dengue-associated HLH diagnosis is challenging, but it is very important to be recognized, as early recognition is associated with better outcomes. Physicians must work in collaboration with pathologists and microbiologists for the proper diagnosis.