acid-phosphatase and Sezary-Syndrome

Topics: Acid Phosphatase; Dermatitis, Exfoliative; Esterases; Glucuronidase; Glycogen; Humans; Lymphocytes; Microscopy, Electron; Mycosis Fungoides; Reticulocytes; Sezary Syndrome; Skin Neoplasms

A cytochemical study in samples from 100 lymphoid leukaemias, 84 of B-cell type and 16 of T-cell type, was carried out with three acid hydrolases: DAP IV, acid phosphatase (AP) and alpha-naphthyl acetate esterase (ANAE). DAP IV was studied in leukaemic T-cells both by cytochemistry and by a monoclonal antibody with the immunoperoxidase technique. Both methods showed similar results. AP and ANAE gave weak reactions in immature B-cell leukaemias (common-ALL and B-CLL) and were strongly expressed in plasma cell disorders. DAP IV showed no activity in any of the types of B-cell leukaemia studied and was strongly positive in some T-cell leukaemias but with a more restricted distribution than ANAE and AP. T-lymphoblasts (T-ALL) and mature (T8+) leukaemias were DAP IV negative. Within the T4+ malignancies DAP IV was positive in four T-prolymphocytic leukaemias, one of two T-CLL and one of three Sezary syndrome cases. Although DAP IV is strictly T-cell specific it does not appear to aid the differentiation between B- and T-cell disorders or the identification of T-cell subsets as determined by monoclonal antibodies. It remains to be established whether this enzyme will define a functionally distinct T-cell subset.

Topics: Acid Phosphatase; B-Lymphocytes; Dipeptidyl Peptidase 4; Dipeptidyl-Peptidases and Tripeptidyl-Peptidases; Histocytochemistry; Humans; Immunoenzyme Techniques; Leukemia; Leukemia, Lymphoid; Naphthol AS D Esterase; Sezary Syndrome; T-Lymphocytes

This paper reports the results of a series of light and electron microscopic, cytochemical, immunologic and cytogenetic investigations performed in a patient with Sézary syndrome. Fifty-two percent of the cerebriform cells were OKIa-1 positive and 55% were acid alpha-naphthyl-acetate esterase (ANAE) negative. Since activated T-lymphocytes are known to lose their peculiar ANAE activity while acquiring Ia-like antigens, it is conceivable that the ANAE-negative and OKIa-1-positive cells represent an in vivo activated subset. Thus, it appears that a typical Sézary cell population from a given individual can exhibit heterogeneous phenotypic profiles.

Topics: Acid Phosphatase; Aged; Chromosome Aberrations; Female; Histocytochemistry; Humans; Naphthol AS D Esterase; Phenotype; Sezary Syndrome

Leukemic blood cells from eight patients with Sézary's syndrome were analyzed for enzyme cytochemical features, Fc receptors, and surface phenotype. Enzyme cytochemically the cases were heterogeneous in their activity of acid esterase, acid phosphatase and dipeptidylaminopeptidase IV (DAP IV). Only one case showed positive staining for DAP IV. The expression of Fc receptors for IgG and IgM also varied. The DAP IV-positive case exhibited Fc mu receptors. In contrast, four other cases showed only Fc gamma receptors. Analysis of the surface antigen pattern of Sézary cells with monoclonal antibodies revealed the phenotype of helper T lymphocytes (Leu-3 a/OKT4+) in all but one case. The Leu-3 a/OKT4-negative case showed a phenotypic feature of natural killer cells (Leu-7+). The results obtained with the antibodies TU14 and Anti-human Lyt-1 were more heterogeneous. The heterogeneity of Sézary cells may be interpreted as a sign of differences in functional differentiation or of proliferation of different T-cell subclones.

Topics: Acid Phosphatase; Animals; Antibodies, Monoclonal; Antigens, Surface; Dipeptidyl-Peptidases and Tripeptidyl-Peptidases; Erythrocytes; Histocytochemistry; Humans; Naphthol AS D Esterase; Phenotype; Receptors, Fc; Receptors, Immunologic; Rosette Formation; Sezary Syndrome; Sheep; T-Lymphocytes

The characteristic large cells in the blood of a patient with Sézary syndrome underwent immunocytological and cytochemical changes during polychemotherapy, which caused transient regression of skin lesions. Tartrate-resistant acid phosphatase was demonstrable in a few cells only after chemotherapy; initially, only 2% T cells and 26% B cells could be demonstrated in the blood by immunocytological methods; after cytostatic therapy, 35% T cells but no B cells were detected.

Topics: Acid Phosphatase; B-Lymphocytes; Cell Nucleus; Cyclophosphamide; Fluocortolone; Humans; Male; Middle Aged; Sezary Syndrome; T-Lymphocytes; Vincristine

Topics: Acid Phosphatase; Cell Nucleus; Diagnosis, Differential; Esterases; Glucuronidase; Humans; Leukemia, Lymphoid; Sezary Syndrome; T-Lymphocytes

Topics: Acid Phosphatase; Esterases; Histocytochemistry; Humans; Leukemia, Lymphoid; Microscopy, Electron; Sezary Syndrome; T-Lymphocytes

Isoenzymatic study of leucocytic acid phosphatase under normal conditions identifies 3 isoenzymatic bands, which exhibit a noticeable cell specificity. Band 2 is granulocytic, band 3 lymphocytic and band 4 monocytic in origin. Pathologic deviations in the isoenzymatic pattern are both qualitative and quantitative. For some diseases such as chronic lymphocytic leukaemia there is a well-defined, differential pattern according to immunological B- or T-cell origin. The more significant qualitative aspects are related to the appearance of abnormal bands, especially band 3b, indicating blastic cellularity, and 5, corresponding to hairy cells. The isoenzymatic analysis of acid phosphatase activity is a simple haematologic complementary test, particularly useful in the differential diagnosis of lymphoproliferative disorders with peripheral blood manifestations.

Topics: Acid Phosphatase; B-Lymphocytes; Diagnosis, Differential; Electrophoresis, Cellulose Acetate; Humans; Isoenzymes; Leukemia; Leukemia, Hairy Cell; Leukemia, Lymphoid; Leukocytes; Lymphocytes; Lymphoma, Non-Hodgkin; Sezary Syndrome; T-Lymphocytes

Tartrate-resistant acid phosphatase has been known to be of diagnostic value in hairy cell leukemia. However, occasionally neoplastic cells of other varieties of lymphoproliferative disorders may contain tartrate-resistant acid phosphatase. The authors have studied four patients with Sézary syndrome who had typical cutaneous lesions with extensive lymphoid infiltrates and circulating atypical E-rosetting lymphoid cells. The abnormal Sézary cells accounted for 23-69% of the peripheral mononuclear cells and often showed convoluted or folded nuclei. These cells in all four patients were strongly positive for acid phosphatase resistant to tartaric acid inhibition. Enzymatic cytochemical studies for acid phosphatase with and without tartrate may be helpful in the differential diagnosis of cutaneous T-cell lymphomas from variants of chronic dermatitis.

Topics: Acid Phosphatase; Electrophoresis, Polyacrylamide Gel; Female; Humans; Male; Middle Aged; Sezary Syndrome; Skin; Tartrates