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.