Peripheral/post-thymic T-cell lymphomas: a spectrum of disease. Clinical, pathologic, and immunologic features of 78...

The clinical, pathologic, and immunologic features of 78 cases of peripheral/post-thymic T-cell lymphomas are described. These neoplasms were extremely heterogeneous and were classified as small lymphocytic, mixed small and large cell, large cell, lymphoepithelioid cell, angiocentric, and adult T-cell leukemia/lymphoma type. Some cases revealed angioimmunoblastic or Hodgkin's-like features. These neoplasms mainly affected older adults (mean age, 57 years; median age, 60 years). Lymphadenopathy represented the most frequent clinical presentation, although most patients demonstrated both nodal (87%) and extranodal involvement (77%) during the course of disease. Sites of extranodal disease included skin/soft tissue, spleen, lung, liver, bone, gastrointestinal tract, central nervous system, peripheral blood, nasopharynx, and retrovaginal tissue. Splenomegaly at presentation was most frequently observed in lymphoepithelioid cell lymphomas. Angiocentric lymphomas involved lung. A mediastinal presentation was typically observed in young adults and associated with a poor prognosis. Patients with gastrointestinal lymphomas presented with bleeding and/or malabsorption. B symptoms were present in most cases (65%). Hypercalcemia occurred in four patients. Phenotypic studies of T-cell antigens demonstrated the loss of one or more pan-T-cell markers in eight of 47 cases evaluated. Assessment of T-cell subsets revealed a helper/inducer phenotype for nearly all immunoreactive cases. For the overall series, 32 patients died of disease (median survival time, 11.5 mo). There was a statistical difference between the combined groups of small lymphocytic and lymphoepithelioid cell types as compared with mixed and large cell types, with a poorer survival for the latter group. Angiocentric and adult T-cell leukemia/lymphoma were associated with poor survival. This series of T-cell lymphomas further documents the marked heterogeneity of this group of neoplasms as well as the poor prognosis observed for certain histologic types.