Case of leukemic changes in hepatosplenic diffuse large B-cell lymphoma (DLBCL) in a human T lymphotropic virus type 1 (HTLV-1) antibody-positive, HCV-negative patient

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A 78-year-old female developed lymphocytosis and splenomegaly. Antibodies to human T-lymphotropic virus type 1 (HTLV-1) were positive; however, incorporation of HTLV-1 proviral DNA was negative. Hepatitis B virus surface antigen (HBsAg) and hepatitis C virus (HCV) antibody were negative. Flow cytometry of the lymphocytes revealed the B-cell phenotype, and rearrangement of the immunoglobulin heavy chain gene was positive. She was treated with cladribine administration and splenic radiation, which were ineffective and she died a month later. Autopsy liver findings revealed infiltration of atypical lymphocytes, which were immunohistochemically positive for CD20, while the spleen revealed diffuse infiltration of atypical lymphocytes which were positive for CD20, Bcl-2 and cytoplasmic c Igk. Some of them were positive for MIB-1 and MUM1. These results indicated leukemic changes in a hepatosplenic diffuse large B-cell lymphoma (DLBCL) with plasma cell differentiation. Lymphoma cells were negative for EBV-encoded latent membrane protein 1 (LMP-1) and EB nuclear antigen-2 (EBNA2) by immunohistochemistry and for EBV-encoded mRNA 1 (EBER-1) by in situ hybridization. The cause that may have contributed to the pathogenesis of hepatosplenic DLBCL is unknown.
Yamaguchi University School of Medicineの論文