Melioidosis complicating hemophagocytic lymphohistiocytosis: A rare diagnostic entity in paediatric
- Department of Paediatrics, School of Medical Sciences, Universiti Sains Malaysia, 16150 Kota Bharu, Kelantan, Malaysia
- Hospital Pakar Universiti Sains Malaysia (HPUSM), Health Campus, Kubang Kerian, 16150 Kota Bharu, Kelantan, Malaysia
- Primary Immunodeficiency Diseases Group, Department of Clinical Medicine, Advanced Medical and Dental Institute, Universiti Sains Malaysia, Bertam, Pulau Pinang, Kepala Batas, 13200, Malaysia
Abstract
Background: Hemophagocytic lymphohistiocytosis is a syndrome characterized by excessive immune activation leading to severe systemic inflammation. It encompasses primary and secondary forms, with the latter often triggered by infections. Paediatric cases of melioidosis complicated by hemophagocytic lymphohistiocytosis are rare and diagnostically challenging.
Case presentation: We report the case of a 7-year-old girl who developed a fever for two months that was unresponsive to antibiotics. She was ultimately diagnosed with melioidosis-associated hemophagocytic lymphohistiocytosis after one month in the ward. She presented with multiorgan involvement, including bicytopenia, severe transaminitis, and neurological symptoms. Prompt initiation of immunoglobulin and steroids led to clinical improvement and resolution of the fever. Diagnostic confirmation relied on meeting HLH-2004 criteria despite limited genetic testing availability.
Conclusion: This case underscores the importance of early recognition and aggressive management in pediatric hemophagocytic lymphohistiocytosis secondary to melioidosis to mitigate potentially fatal outcomes.