Hepato-splenic gamma delta T-cell lymphoma: A rare entity mimicking the hemophagocytic syndrome

Hepatosplenic gamma delta T-cell lymphoma is a rare histologic type of the peripheral T-cell lymphomas, clinically characterized by predominant involv ement of liver and spleen, no or little adenopathy, and an often aggressive course; it affects mainly adolescents and young adults, with a male predom inance. Postthymic T-cell malignancies are heterogeneous in their clinical and laboratory features. Among the gamma delta postthymic T-cell lymphomas, two distinct entities (cutaneous and hepatosplenic, respectively) are repo rted in the literature, The former shows predominant multiple involvement o f the skin and subcutaneous tissue; it occurs mostly in older patients and the phenotype is CD3-, CD4-, CD8-. Because of the small number of reports, the course of the disease was unknown. The latter shows a clinical picture characterized by hepatosplenomegaly, no or little adenopathy, and sometimes systemic symptoms (fever, cytopenias likely due to hypersplenism); it pres ents a peculiar sinusoidal involvement of liver and spleen. The bone marrow histologic feature often reveals a little infiltration, especially sinusoi dal and easily underestimated phenotype: CD2+, CD3+, CD7+, CD5-, CD4-, CD8- , CD44+, Few cases of this lymphoma associated by hemophagocytic syndrome a re described (Sun, 1990; Kadin, 1981; Jaffe, 1983), We report a case of a y oung man with a rapid and fatal course in which the more important clinical feature was hemophagocytosis. The diagnosis of lymphoma was very difficult because of paucity of histologic involvement, and only the rearrangement o f TCR gamma chain gene by polymerase chain reaction on paraffin sections co nfirmed a clonal T-cell proliferation, (C) 1999 Wiley-Liss, Inc.