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Nonhepatosplenic   T-Cell Lymphoma: A Subset of Cytotoxic
Lymphomas With Mucosal or Skin Localization
Bertrand Arnulf,
Christiane Copie-Bergman,
Marie-Hélène Delfau-Larue,
Anne Lavergne-Slove,
Jacques Bosq,
Janine Wechsler,
Michel Wassef,
Claude Matuchansky,
Bernard Epardeau,
Marc Stern,
Martine Bagot,
Felix Reyes, and
Philippe Gaulard
From the Département de Pathologie and EA2348, Service
d'Immunologie Biologique, CHU Henri Mondor, Créteil;
Département de Pathologie, Hôpital Lariboisière,
Paris; Département de Pathologie, Institut Gustave Roussy,
Villejuif; Service de Gastroentérologie, Hôpital Saint
Lazare, Paris; Département de Pathologie, Service de Pneumologie,
Hôpital Foch, Suresnes; Service de Dermatologie and Groupe
d'étude Français des Lymphomes Cutanés; and Service
d'Hématologie Clinique, CHU Henri Mondor, Créteil, France.
Human   T lymphocytes represent a minor subset of T cells in
the peripheral blood, which exhibit a limited diversity and a
tissue-restricted repertoire in contrast to their broad specificity. Most postthymic neoplasms that arise from this T-cell subpopulation belong to the hepatosplenic lymphoma entity. Only a few cases of
nonhepatosplenic lymphomas have been described in detail previously. This study presents the clinicopathologic features of 11 consecutive cases of nonhepatosplenic lymphoma. All were characterized by mucosal or skin initial involvement: nasal cavity (n = 3), gastrointestinal tract (n = 3), skin (n = 3), lung
(n = 1), larynx (n = 1). Most patients presented with B
symptoms (eight of 11), without peripheral lymphadenopathy and bone
marrow involvement. A past history of chronic antigen exposure was
noted in six cases, and four patients had features of immune
deficiency. On histology, they were classified as pleomorphic tumors.
Features of epitheliotropism and angiocentrism was observed in most
cases. Tumor cells had a CD2+, CD3+, T-cell
receptor (TCR) 1+,  F1 phenotype.
They were CD5 (9 of 10) and
CD4/CD8 (9 of 10) or CD8+
(1 of 10). A clonal -chain gene rearrangement was detected in all
tested cases (9/9). All cases had an activated cytotoxic T-cell intracellular antigen-1 (TIA-1)+, Granzyme
B+ phenotype. Epstein-Barr virus (EBV) sequences were
detected in six cases by in situ hybridization (ISH). Despite an
aggressive clinical course, complete remission was obtained in three
patients, and one of the latter required a peripheral blood stem-cell
transplantation. Nonhepatosplenic peripheral T-cell lymphoma can
be regarded as a model of activated cytotoxic lymphoma, occurring in
mucosae or skin. These appear to be derived from the subpopulation of tissue-restricted lymphocytes, which are involved in the host epithelial surface surveillance. The role of chronic antigen exposure in the pathogenesis of these rare lymphomas can be suggested, in view
of the past history observed in at least some patients.
Blood, Vol. 91 No. 5 (March 1), 1998:
pp. 1723-1731
© 1998 by The American Society of Hematology.
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