The Sézary syndrome: a malignant proliferation of helper T cells. 1976

S Broder, and R L Edelson, and M A Lutzner, and D L Nelson, and R P MacDermott, and M E Durm, and C K Goldman, and B D Meade, and T A Waldmann

The Sézary syndrome is a frequently lethal disease characterized by circulating malignant cells of thymus-derived (T)-cell origin. The capacity of circulating malignant lymphocytes from patients with this syndrome to synthesize immunoglobulins and to function as helper or suppressor cells regulating immunoglobulin synthesis by bone marrow-derived (B) lymphocytes was determined. Peripheral blood lymphocytes from normal individuals had geometric mean immunoglobulin synthetic rates of 4,910 ng for IgM, 1,270 ng for IgA, and 1,625 ng for IgG per 2 X 10(6) cells in culture with pokeweed mitogen for 7 days. Purified normal B cells had geometric mean synthetic rates of 198 ng for IgM, 145 ng for IgA, and 102 ng for IgG. Leukemic cells from patients with the Sézary syndrome produced essentially no immunoglobulins. Adding normal T cells to normal B cells restored their immunoglobin producing capacity. Leukemic cells from four of five patients tested had a similar capacity to help immunoglobulin synthesis by purified normal B cells. Additionally, Sézary cells from one patient studied induced a nearly 10-fold increase in IgA synthesis by lymphocytes from a child with ataxia telangiectasia and selective IgA deficiency. Furthermore, these Sézary cells induced more than a 500-fold increase in IgG and IgA synthesis by lymphocytes from a child with Nezelof's syndrome. When Sézary cells were added to normal unfractionated lymphocytes, they did not suppress immunoglobulin biosynthesis. In addition, unlike the situation observed when large numbers of normal T cells were added to purified B cells, there was no depression of immunoglobulin synthesis at very high malignant T-cell to B-cell ratios. These data support the view that Sézary T cells do not express suppressor cell activity. The results presented in this paper suggest that neoplastic lymphocytes from the majority of patients with the Sézary syndrome originate from a subset of T cells programmed exclusively for helper-like interactions with B cells in their production of immunoglobulin molecules.

UI MeSH Term Description Entries
D007136 Immunoglobulins Multi-subunit proteins which function in IMMUNITY. They are produced by B LYMPHOCYTES from the IMMUNOGLOBULIN GENES. They are comprised of two heavy (IMMUNOGLOBULIN HEAVY CHAINS) and two light chains (IMMUNOGLOBULIN LIGHT CHAINS) with additional ancillary polypeptide chains depending on their isoforms. The variety of isoforms include monomeric or polymeric forms, and transmembrane forms (B-CELL ANTIGEN RECEPTORS) or secreted forms (ANTIBODIES). They are divided by the amino acid sequence of their heavy chains into five classes (IMMUNOGLOBULIN A; IMMUNOGLOBULIN D; IMMUNOGLOBULIN E; IMMUNOGLOBULIN G; IMMUNOGLOBULIN M) and various subclasses. Globulins, Immune,Immune Globulin,Immune Globulins,Immunoglobulin,Globulin, Immune
D008206 Lymphatic Diseases Diseases of LYMPH; LYMPH NODES; or LYMPHATIC VESSELS. Lymphatism,Status Lymphaticus,Disease, Lymphatic,Diseases, Lymphatic,Lymphatic Disease
D008297 Male Males
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D009360 Neoplastic Cells, Circulating Exfoliate neoplastic cells circulating in the blood and associated with metastasizing tumors. Circulating Neoplastic Cells,Embolic Tumor Cells,Embolism, Tumor,Neoplasm Circulating Cells,Tumor Cells, Embolic,Cells, Neoplasm Circulating,Circulating Cells, Neoplasm,Circulating Tumor Cells,Cell, Circulating Neoplastic,Cell, Circulating Tumor,Cell, Embolic Tumor,Cell, Neoplasm Circulating,Cells, Circulating Neoplastic,Cells, Circulating Tumor,Cells, Embolic Tumor,Circulating Neoplastic Cell,Circulating Tumor Cell,Embolic Tumor Cell,Embolisms, Tumor,Neoplasm Circulating Cell,Neoplastic Cell, Circulating,Tumor Cell, Circulating,Tumor Cell, Embolic,Tumor Cells, Circulating,Tumor Embolism,Tumor Embolisms
D003873 Dermatitis, Exfoliative The widespread involvement of the skin by a scaly, erythematous dermatitis occurring either as a secondary or reactive process to an underlying cutaneous disorder (e.g., atopic dermatitis, psoriasis, etc.), or as a primary or idiopathic disease. It is often associated with the loss of hair and nails, hyperkeratosis of the palms and soles, and pruritus. (From Dorland, 27th ed) Dermatitis Exfoliative,Dermatitis Exfoliative Generalised,Dermatitis Exfoliative Generalized,Erythroderma,Dermatitis Exfoliativa,Dermatitis Exfoliative Generaliseds,Dermatitis Exfoliatives,Erythrodermas,Exfoliative Dermatitides,Exfoliative Dermatitis,Exfoliative Generalised, Dermatitis,Exfoliative Generalized, Dermatitis,Exfoliative, Dermatitis,Exfoliatives, Dermatitis,Generalised, Dermatitis Exfoliative
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults
D000368 Aged A person 65 years of age or older. For a person older than 79 years, AGED, 80 AND OVER is available. Elderly

Related Publications

S Broder, and R L Edelson, and M A Lutzner, and D L Nelson, and R P MacDermott, and M E Durm, and C K Goldman, and B D Meade, and T A Waldmann
February 1983, Clinical immunology and immunopathology,
S Broder, and R L Edelson, and M A Lutzner, and D L Nelson, and R P MacDermott, and M E Durm, and C K Goldman, and B D Meade, and T A Waldmann
September 1983, Nihon Naika Gakkai zasshi. The Journal of the Japanese Society of Internal Medicine,
S Broder, and R L Edelson, and M A Lutzner, and D L Nelson, and R P MacDermott, and M E Durm, and C K Goldman, and B D Meade, and T A Waldmann
May 2016, Acta dermato-venereologica,
S Broder, and R L Edelson, and M A Lutzner, and D L Nelson, and R P MacDermott, and M E Durm, and C K Goldman, and B D Meade, and T A Waldmann
April 1983, The American Journal of dermatopathology,
S Broder, and R L Edelson, and M A Lutzner, and D L Nelson, and R P MacDermott, and M E Durm, and C K Goldman, and B D Meade, and T A Waldmann
February 2008, The Journal of investigative dermatology,
S Broder, and R L Edelson, and M A Lutzner, and D L Nelson, and R P MacDermott, and M E Durm, and C K Goldman, and B D Meade, and T A Waldmann
September 1978, Blood,
S Broder, and R L Edelson, and M A Lutzner, and D L Nelson, and R P MacDermott, and M E Durm, and C K Goldman, and B D Meade, and T A Waldmann
September 2013, Journal of the American Academy of Dermatology,
S Broder, and R L Edelson, and M A Lutzner, and D L Nelson, and R P MacDermott, and M E Durm, and C K Goldman, and B D Meade, and T A Waldmann
August 2019, Oncotarget,
S Broder, and R L Edelson, and M A Lutzner, and D L Nelson, and R P MacDermott, and M E Durm, and C K Goldman, and B D Meade, and T A Waldmann
January 1982, Acta dermato-venereologica,
S Broder, and R L Edelson, and M A Lutzner, and D L Nelson, and R P MacDermott, and M E Durm, and C K Goldman, and B D Meade, and T A Waldmann
February 1998, Leukemia & lymphoma,
Copied contents to your clipboard!