Hodgkin Lymphoma in a Child Presenting as HLH (Haemophagocytic Lymphohistiocytosis)-EBV is Central to Both Entities: A Case Report and Review of Literature

Abstract

Abstract: HLH is a rare life threatening systemic disease resulting from unregulated immune activation presenting with diverse clinical manifestations and etiologies. While Primary or familial HLH is a relatively common occurrence in infants and young children, secondary HLH due to infections, malignancy, rheumatologic and autoimmune disorders is infrequent and carries a grave prognosis owing to delayed or misdiagnosis. Albeitits rarity in children, EBV infection remains the leading cause of both Hodgkin's lymphoma and HLH worldwide. Additionally, data for simultaneous presence of these three entities at the time of diagnosis is limited. Our report focuses on one such case where a patient's clinical signs and symptoms were non-specific and masked by previous history of idiopathic HLH. After being in remission for 2-3 months patient again relapsed but this time around the etiology was Hodgkin's lymphoma with an underlying EBV infection.