International Journal of Preclinical and Clinical Research

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is an autoimmune phenomenon characterized by reactive hyperactivity of cytotoxic T cells and histiocytes, leading to hypercytokinemic injury to cells and organ system, which leads to multiorgan dysfunction and ultimate failure. Epstein-Barr virus (EBV) is most commonly associated with secondary HLH with high mortality, but increasing evidence suggests the association of the dengue virus. When associated with dengue infection, it carries a grave prognosis and correlates with the disease severity. Furthermore, it overlaps with dengue sepsis, so it can often be misdiagnosed as sepsis. Typically, the patients have hyperferritinemia, hypertriglyceridemia, transaminitis, and marrow features suggestive of hemophagocytosis. The treatment is usually systemic corticosteroids, intravenous immunoglobulin, and chemotherapy with etoposide. 30 years old male presented to our OPD with complaints of fever for 4-5 months on and off type and generalised weakness. Clinical suspicion alerted us to look for other evidence of HLH on the fourth day of admission, and appropriate investigations were done. Diagnosis of HLH was confirmed by HLH-2004, HScore criteria, and bone marrow aspirate examination. Treatment was given in the form of corticosteroids and chemotherapy along with other supportive measures (dexamethasone). The patient had cardiac arrest and succumbed inspite of necessary measures taken to rescussitate. HLH is life threatening complication. The treatment of this condition requires aggressive immunosuppression with either the cytotoxic agents or anti T cell antibodies.

Keywords: Hemophagocytosis, HLH­2004, H Score Criteria, ETOPOSIDE, Cytokine Storm