Secondary Hemophagocytic Lymphohistiocytosis - A Common Ramification of Different Diseases

Hemophagocytic lymphohistiocytosis is a rare and potentially fatal disorder caused by immune dysregulation. It can occur as a primary genetic disease or secondarily due to various causes including infections, malignancies or autoimmune diseases. In this case report, we present two cases of Hemophago...

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Bibliographic Details
Published in:EJIFCC 2022-04, Vol.33 (1), p.63-69
Main Authors: Gnanasambandam, Janani, Geminiganesan, Sangeetha, Vilvanathan, Vairan, Sankaranarayanan, Shuba, Padmanabhan, Ramachandran
Format: Article
Language:English
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Case Study
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Summary:Hemophagocytic lymphohistiocytosis is a rare and potentially fatal disorder caused by immune dysregulation. It can occur as a primary genetic disease or secondarily due to various causes including infections, malignancies or autoimmune diseases. In this case report, we present two cases of Hemophagocytic lymphohistiocytosis which were secondary to typhoid and dengue fever. While primary disease occurs predominantly in infants, secondary hemophagocytic lymphohistiocytosis can occur in any age group. Both primary and secondary hemophagocytic lymphohistiocytosis are characterised by fever, hepatosplenomegaly, pancytopenia and multiorgan dysfunction. But unusual persistence of fever and other organ involvement should need further workup for hemophagocytic lymphohistiocytosis. Secondary hemophagocytic lymphohistiocytosis may resolve on treating the underlying disorder. But severe cases need treatment with immunosuppressive/immunomodulation therapy to prevent morbidity. Early clinical suspicion, prompt diagnosis and treatment of hemophagocytic lymphohistiocytosis are essential to prevent deleterious effects to health.
ISSN:1650-3414