CASE REPORT

Hepatosplenic T cell lymphoma with no expression of cytotoxic molecules

Y Sadahira¹, K Notohara² and T Manabe¹

¹ Department of Pathology, Kawasaki Medical School, Kurashiki 701–0192, Japan
² Department of Pathology, Okayama Medical School, Okayama 700-8558, Japan

Correspondence to:
Correspondence to:
Dr Y Sadahira, Department of Pathology, Kawasaki Medical School, 577 Matsushima, Kurashiki 701–0192, Japan;
sadapath{at}med.kawasaki-m.ac.jp

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, CD8, and CD45RO and clonal rearrangement of the TCR gene by the polymerase chain reaction on paraffin wax embedded sections. However, they were negative for TIA-1 and granzyme B, in addition to ßF1, 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.

Keywords: cytotoxic; hepatosplenic; T cell lymphoma

Abbreviations: ALT, alanine aminotransferase; AST, aspartate aminotransferase; EBER1, Epstein-Barr virus encoded mRNA 1; EBV, Epstein-Barr virus; HB, hepatitis B; HPTL, hepatosplenic T cell lymphoma; LDH, lactate dehydrogenase; NK, natural killer; TIA-1, T cell intracellular antigen

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