Terminal deoxynucleotidyl transferase positive lymphoblastic lymphoma: a study of 15 cases.

The investigation was undertaken to define the features of lymphoblastic lymphoma. Fifteen lymph node biopsies from a group of 82 specimens studied for the enzyme terminal deoxynucleotidyl transferase (TdT) fulfilled morphological criteria for this diagnosis. These criteria required a diffuse infiltrate of relatively uniform, immature lymphoid cells with basophilic cytoplasm; round, oval or lobulated nuclei with evenly dispersed chromatin; rare or inconspicuous nucleoli; and numerous mitotic figures. Examination of 1-micron thick, plastic-embedded, Giemsa-stained tissue sections revealed convoluted nuclei in more than 50% of neoplastic cells in four cases: in six specimens there was an admixture of cells with grooved, hyperlobulated, and round nuclei, and in five the round or oval nuclei were non-convoluted. Specimens from all 15 patients were positive for TdT by fluorescent antibody and biochemical assays. The percentage of cells from involved nodes reacting by indirect immunofluorescence with an antiserum against bovine TdT ranged from 4 to 90% (mean of 52%), and the mean level of biochemically measured enzyme activity was 8.7 units/g of tissue (range of 1.9 to 27.5). Cytochemical stains for acid phosphatase were positive in 13 of the 15 cases. In eight samples more than 50% of cells formed rosettes with sheep erythrocytes, while the E rosettes varied from 14 to 38% in the other seven. The percentage of cells with complement receptors varied widely (range of 6 to 80), but cells bearing surface immunoglobulin or IgGfc receptors were not increased. All patients presented with supradiaphragmatic lymphaedenopathy, eight with an anterior mediastinal mass. Two-thirds of the patients were male, and the mean age was 20 years (range 4 to 46 years). None were leukemic at the time of diagnosis, but eight patients subsequently developed acute lymphoblastic leukemia. Involvement of the central nervous system was observed in four of the 15, and of the testes in two. Ten patients have died of their disease with a median survival of 8 months (range 4 to 20), and five are alive 3--8 months after diagnosis. We observed no differences in clinical findings at presentation, incidence of mediastinal involvement or leukemic dissemination, content of TdT, acid phosphatase staining, or immunologic cell surface characteristics between the convoluted and non-convoluted types of lymphoblastic lymphoma. Distinctive morphologic, cell surface, biochemical, and clinical features of lymphoblastic lymphoma can be identified irrespective of the presence or absence of convoluted nuclei.