title: Hepatosplenic T cell lymphoma with no expression of cytotoxic molecules.
abstract: Hepatosplenic T cell lymphoma is defined as an extranodal and systemic neoplasm derived from cytotoxic T cells. This report describes a postmortem case of T cell lymphoma that showed histological features of hepatosplenic T cell lymphoma but did not express cytotoxic molecules. The patient was a 57 year old man who presented with severe icterus and hepatosplenomegaly, followed by an aggressive clinical course. The liver and spleen were enlarged, weighing 2000 g and 360 g, respectively. Histologically, the liver, spleen, and bone marrow were entirely affected by lymphoma, comprising pleomorphic small and large cells, which displayed sinusoidal infiltration in the liver, diffuse infiltration in the splenic cord, and interstitial/diffuse infiltration with fibrosis in the bone marrow. Lymphoma cells showed positivity for CD3 epsilon, CD8, and CD45RO and clonal rearrangement of the TCRgamma gene by the polymerase chain reaction on paraffin wax embedded sections. However, they were negative for TIA-1 and granzyme B, in addition to betaF1, CD4, and CD56. Few neoplastic cells were stained for Epstein-Barr virus encoded mRNA 1. These findings indicate that this case might represent a variant of hepatosplenic T cell lymphoma despite the absence of cytotoxic molecules.
authors.affiliation: Department of Pathology, Kawasaki Medical School, Kurashiki 701-0192, Japan.
authors.forename: Y
authors.initials: Y
authors.lastname: Sadahira
authors.forename: K
authors.initials: K
authors.lastname: Notohara
authors.forename: T
authors.initials: T
authors.lastname: Manabe
mesh_terms: D014408:Biomarkers, Tumor / Q000032:analysis*; D017252:CD3 Complex; D016827:CD8 Antigens / Q000032:analysis; D018414:CD8-Positive T-Lymphocytes / Q000737:chemistry*; D003602:Cytotoxicity, Immunologic; D015334:Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor; D053804:Granzymes; D006801:Humans; D007124:Immunoenzyme Techniques; D017493:Leukocyte Common Antigens / Q000032:analysis; D008113:Liver Neoplasms / Q000235:genetics / Q000473:pathology*; D016399:Lymphoma, T-Cell / Q000235:genetics / Q000473:pathology*; D008297:Male; D008565:Membrane Proteins / Q000032:analysis*; D008875:Middle Aged; D039102:Poly(A)-Binding Proteins; D011506:Proteins; D016601:RNA-Binding Proteins / Q000032:analysis*; D011948:Receptors, Antigen, T-Cell / Q000032:analysis; D012697:Serine Endopeptidases / Q000032:analysis; D013160:Splenic Neoplasms / Q000235:genetics / Q000473:pathology*; D000074019:T-Cell Intracellular Antigen-1
chemical_list: D014408:Biomarkers, Tumor; D017252:CD3 Complex; C115335:CD3E protein, human; D016827:CD8 Antigens; D008565:Membrane Proteins; D039102:Poly(A)-Binding Proteins; D011506:Proteins; D016601:RNA-Binding Proteins; D011948:Receptors, Antigen, T-Cell; D000074019:T-Cell Intracellular Antigen-1; C071558:TIA1 protein, human; D017493:Leukocyte Common Antigens; C509213:GZMB protein, human; D053804:Granzymes; D012697:Serine Endopeptidases