EBV-positive peripheral T-cell lymphoma with extensive hemophagocytosis.

A 46-year-old man with HIV was admitted with subacute onset of fever, cough, generalized weakness, and weight loss. He went on to develop multiorgan failure with encephalopathy, acute kidney injury, fulminant liver failure, and disseminated intravascular coagulation. He was also found to have splenomegaly, pancytopenia, and cavitary lesions in the lungs at presentation. Further workup showed hyperferritinemia (.90 000 mg/L), hypertriglyceridemia (782 mg/dL), and Epstein-Barr virus (EBV) viremia (61 200 copies/mL). The diagnostic criteria for hemophagocytic lymphohistiocytosis (HLH) were met. Bone marrow biopsy showed extensive hemophagocytosis (panel A, black arrows). The infiltrating neoplastic cells were intensely positive for CD8 immunostaining, suggesting cytotoxic phenotype (panel B), and EBV RNA by in situ hybridization (panel C). Cytogenetic analysis showed complex karyotype and the T-cell gene receptor was clonally rearranged. The final diagnosis was HIV-associated EBV-positive peripheral T-cell lymphoma of cytotoxic phenotype with extensive hemophagocytosis.