Leukemic reticuloendotheliosis: "hairy cell leukemia," functional and structural features of the abnormal cell in a patient with profound leukocytosis.
The development of profound leukocytosis in a patient with leukemic reticuloendotheliosis (LRE) enabled us to obtain purified LRE cells for the investigation of their structural and functional characteristics. The LRE cells of our patient bore surface immunoglobulin and had complement receptors but did not bear Fc receptors and did not form rosettes with sheep erythrocytes. By electron microscopy, the cells were observed to contain typical ribosome lamella structures and to phagocytize both 0.81 micron latex particles and complement-coated zymosan particles. They were adherent to both glass and nylon wool fibers. The mitogenic response to erythroagglutinating phytohemagglutinin was normal to magnitude but delayed chronologically. The binding of 125I-labeled plant lectins was used to characterize the surface topography of LRE cells. Results of these studies indicated that the LRE cell surface differed significantly from the surface of normal T and B lymphocytes and chronic lymphatic leukemia cells. The LRE cells were capable of both stimulating and responding in a one-way mixed lymphocyte culture. However, the LRE cells were not active as effector cells of either cell-mediated lympholysis, a T cell function, or antibody-dependent cellular cytotoxicity, a null cell function. In contrast, they were effector cells of lectin-induced cellular cytotoxicity showing that they did possess the capacity to function as cytotoxic effector cells. These data indicated that the LRE cells in our patient had surface and functional characteristics of both lymphocytes and monocytes.
D001665:Binding Sites; D002448:Cell Adhesion; D003165:Complement System Proteins; D003601:Cytotoxicity Tests, Immunologic; D006644:Histiocytes / Q000473:pathology; D006801:Humans; D007141:Immunoglobulin Fc Fragments; D007938:Leukemia / Q000473:pathology*; D007964:Leukocytosis / Q000150:complications; D008206:Lymphatic Diseases / Q000473:pathology*; D007959:Lymphocyte Culture Test, Mixed; D008214:Lymphocytes / Q000473:pathology; D008297:Male; D008875:Middle Aged; D009000:Monocytes / Q000276:immunology / Q000648:ultrastructure*; D010587:Phagocytosis; D011947:Receptors, Antigen, B-Cell
D007141:Immunoglobulin Fc Fragments; D011947:Receptors, Antigen, B-Cell; D003165:Complement System Proteins