Introduction: Hemophagocytic lymphohistiocytosis (HLH) is a serious syndrome that occurs in association
with malignancy, infections and autoimmune diseases.
Case presentation: A 30 years old female patient presented with fever, splenomegaly, pancytopenia,
hyperferritinemia, hypertriglyceridemia and hemophagocytosis in bone marrow aspirate. She was initially
diagnosed as HLH. Bone marrow biopsy revealed the presence of Hodgkin lymphoma.
Discussion: The most common HLH associated-malignancy is T/natural killer cell lymphoma and less
commonly diffuse large B-cell lymphoma. The combination of HLH and Hodgkin lymphoma is rare. A strong association between Hodgkin lymphoma associated HLH and Epstein Barr virus (EBV) infection has
been discussed in published case reports. Hodgkin lymphoma may be a cause of HLH and their relation with
EBV has to be investigated.