Philippine E-Journals

Dengue-Associated Hemophagocytic Lymphohistiocytosis with Central Nervous System Involvement in a SevenYear-Old Male: A Case Report

Jay Christopher L Yang | Monique M. Yoingco | Ma. Lilybeth R. Tanchoco

Discipline: Medicine

 

Abstract:

Hemophagocytic lymphohistiocytosis (HLH) is a rare disease estimated to occur in 1 of every 3000 children admitted in tertiary centers, and usually manifests as fever with multi-organ involvement. However, the incidence of dengue-associated HLH has not yet been established. Laboratory workup generally makes up the requirements for diagnosis, hence it may remain undiagnosed in lacking facilities. The disease is aggressive with a high mortality rate, as high as 21.6%, especially if not treated early. The prognosis remains relatively good with a 5-year survival rate of more than 50% in patients who receive early HLH treatment, consisting of 8-weeks of etoposide chemotherapy with dexamethasone, and in those with CNS involvement, intrathecal chemotherapy with methotrexate. Patients who have undergone remission will have to undergo regular surveillance for recurrence. HLH is either primary, when there is a background of genetic disease, or secondary, when triggered by infectious causes, including dengue virus, which precipitated the occurrence in our patient. HLH is diagnosed by meeting 5 of the 9 criteria: fever, splenomegaly, cytopenia, hyperferritinemia, hypertriglyceridemia, hypofibrinogenemia, low or absent natural killer cell activity, hemophagocytosis and high-soluble interleukin-2 receptor levels. Our patient was admitted initially as a case of dengue fever. He had fever, splenomegaly, hyperferritinemia, hypertriglyceridemia, and hemophagocytosis, hence the diagnosis of dengue-associated HLH. He initially underwent HLH treatment protocol with etoposide and dexamethasone. He was readmitted due to a seizure episode and underwent intrathecal chemotherapy with methotrexate under general anesthesia (GA) after stabilization and was discharged improved. In an anesthesiologist’s standpoint, intrathecal chemotherapy is challenging in the pediatric population since they are usually uncooperative. Hence the setup is very important since immobility is needed. Pneumonia also imposes another risk of hyperreactive airway in children, making anesthesia complex.