Characterization of two patients with lymphomas of large granular lymphocytes (original) (raw)

Abstract

Two patients with non cutaneous well-differentiated lymphocytic lymphoma with leukemic spread are reported. The large majority of their peripheral blood mononuclear cells (PBMC) formed rosettes with sheep erythrocytes, had receptors for the Fc portion of IgG, and an enzymatic profile of relatively mature T-cells. These cells were morphologically characterized as large granular lymphocytes. Studies with monoclonal antibodies in one of the cases showed an OKT3+, OKTlO-, OKT4-, OKTS-, HNK-I-, OKMl+ phenotype, whereas PBMC from the other case were OKT3+, OKTlO-, OKT4-, OKT8+, HNK-I+, OKMl-. PBMC from the first patient were able to suppress in vitro B-cell differentiation and were capable of a strong antibody dependent cellular cytotoxicity (ADCC) activity. Natural killer (NK) activity was reduced. Cells from the other patient who was hypogammaglobulinemic, exerted suppressor activity in immunoregulatory assays, and showed ADCC and NK activity. These data support the existence of LGL lymphomas consisting of the proliferation of mature appearing cells capable of functional activity. Cancer 53:445-452, 1984.-CELL LYMPHOMAS include a number of disorders T which are heterogeneous with respect to presentation, clinical course, pathology, and characteristics of the proliferating cells. The lymphoblastic subtype represents the most common form of noncutaneous T-cell malignant lymphomas. Proliferating cells in these cases are immature, and the typical presentation is supradiaphragmatic with lymph nodes and thymic involvement. Early on, the tumor spreads to an overt leukemia (identical to childhood T-acute lymphoblastic leukemia [T-ALL]), often with involvement of the central nervous system, and is rapidly fatal.' On the other hand, the rare cases of welldifferentiated lymphocytic lymphoma with leukemia are indistinguishable from chronic lymphocytic leukemia (T

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