Erythrophagocytic T-cell lymphoma: immunologic and cytogenetic evidence for a clonal malignancy of the CD8-positive T-cell subclass.
We report a case of an erythrophagocytic T-cell lymphoma with autoimmune hemolytic anemia, osteolytic lesions, hypercalcemia, hepatomegaly, and marrow invasion without evident lymph node, skin or peripheral blood involvement. The malignant cells were large immunoblastic cells with pleomorphic nuclei and occasional phagocytic vacuoles containing erythrocytes. Immunologic studies showed the tumor cells to have the phenotype of activated T-cells of the cytotoxic/suppressor subclass (HLA- Dr-positive, CD8 positive) with surface receptors for IgG Fc but lacking functional activity in assays of natural killer activity and antibody-dependent cytotoxicity. The tumor cells appeared to express receptors for T-cell growth factor (Tac). Cytogenetic study of marrow mononuclear cells revealed complex but non-random karyotypic abnormalities (45 XX, +11, -12, -16, 17 p+). The clinical and laboratory features indicate that erythrophagocytic T-cell lymphoma represents a true clonal malignancy of the CD8-positive subclass of T-lymphocytes.
D000368:Aged; D000744:Anemia, Hemolytic, Autoimmune / Q000150:complications; D000945:Antigens, Differentiation, T-Lymphocyte; D015415:Biomarkers; D016827:CD8 Antigens; D002869:Chromosome Aberrations; D003602:Cytotoxicity, Immunologic; D004912:Erythrocytes / Q000276:immunology / Q000473:pathology; D005260:Female; D006801:Humans; D008213:Lymphocyte Activation; D008223:Lymphoma / Q000150:complications / Q000235:genetics / Q000276:immunology*; D010586:Phagocytes / Q000276:immunology / Q000473:pathology; D013601:T-Lymphocytes / Q000145:classification / Q000276:immunology
D000945:Antigens, Differentiation, T-Lymphocyte; D015415:Biomarkers; D016827:CD8 Antigens