Large granular lymphocytosisとhemophagocytic syndromeを合併したEpstein-Barrウイルス感染症の1剖検例

An unusual case with Epstein-Barr virus (EBV) infection accompanied by large granular lymphocytosis, hemophagocytic syndrome and aggressive clinical course was described. The phenotype of the large granular lymphocytes in the peripheral blood were as followed: CD2+, CD3-, CD4-, CD8-, CD16+, CD25-, CD45RA+, CD56+, CD57- and HLA-DR+. These cells also showed natural killer activity. The terminal clinicopathological features were quite similar to those of malignant histiocytosis. The autopsy showed extensive proliferation of lymphoid cells and erythrophagocytic macrophages in the almost all organs especially in the spleen (1800g), liver (3300g) and bone marrows. We consider that EBV infection accompanied by large granular lymphocytosis has an important position in hemophagocytic syndrome.