An Unusual Natural Killer-like Hepatosplenic Lymphoma Associated with the Hemophagocytic Syndrome

Abstract

We describe a unique case of a hepatosplenic lymphoma associated with hemophagocytosis. The patient was a 53 yr old man who died rapidly of this disease. Postmortem examination revealed a lymphomatous infiltrate involving primarily the liver, spleen, and bone marrow. The lymphoma was poIymorphous with scattered large, bizarre, anaplastic cells that were CD45R0, CD43, TIA-1, and granzyme B positive but were negative for CD3, CD4, CD5, CD8, CD56, CD57, CD68, CD20, ALK, CD 15, CD I a, S 100, or CD30. Molecular analysis did not reveal a T cell receptor gene rearrangement nor document EBV sequences within the tumor. Heinophagocytosis was prominent in the bone marrow and spleen and was also observed in sections from the liver and lymph node. The clinical and pathologic features of this case do not conform well to any of the welldescribed lymphoma subtypes and are suggestive of a natural killer cell origin of the neoplasm. Related lymphoproliferative disorders will be discussed. (The J Histotechnol 25:23, 2002)

Submitted: March 14, 200 1 ; Accepted with revisions: December 10, 2001

Keywords:

• liver
• neoplastic cells
• tumor cells