Dengue-Induced Hemophagocytic Lymphohistiocytosis: A Case Report and Literature Review

Abstract

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. Dengueassociated 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.

Hemophagocytic lymphohistiocytosis as a rare complication of dengue haemorrhagic fever: a case report

“…At the same time, the patient had a successful response to intravenous dexamethasone therapy for 2 weeks followed by an 8-week tapering regimen. This treatment approach differs from some of the other cases reported in the literature, where different immunosuppressive agents and/or hemopoietic stem cell transplants were used [ 6 ]. Therefore, this case highlights the effectiveness of dexamethasone in managing HLH associated with dengue infection and adds to the existing knowledge on the management of this rare and potentially fatal complication of dengue fever.…”

Hemophagocytic Lymphohistiocytosis Secondary to Dengue Fever in a Pediatric Patient: A Case Report

“…HLH can be either familial (primary) or acquired (secondary). Primary HLH is more common than secondary HLH, with an incidence rate of 1.2 per million children per year [ 3 ]. However, there are currently no comprehensive reports thoroughly characterizing the incidence associated with each etiology of secondary HLH.…”

A Case Report on CNS Hemophagocytic Lymphohistiocytosis in an Infant With Dengue Hemorrhagic Fever

“…It can be secondary when triggered by malignant or non-malignant diseases like juvenile idiopathic arthritis (JIA), systemic lupus erythematosus (SLE), other autoimmune diseases, and infections like Epstein Barr Virus (EBV), human immunodeficiency virus (HIV), cytomegalovirus (CMV), tuberculosis (TB), dengue and possibly severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) [3]. Timely administration of steroids has led to improved outcomes in HLH with dengue [4]. The incidence of HLH with dengue in children has been well-reported [4].…”

“…Timely administration of steroids has led to improved outcomes in HLH with dengue [4]. The incidence of HLH with dengue in children has been well-reported [4]. However, CNS HLH associated with dengue has not been described in the literature yet.…”