The kinetics of Sézary cell production.
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Biomedical subjects
Publications and source records attributed to R Edelson.
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Immunoabsorbent columns, containing membrane fragments of either leukemic "helper" T cells or B cell lymphoblasts, were used to isolate and study antilymphocyte antibodies from plasmas of 2 patients with systemic lupus erythematosus (SLE). Both plasmas contained IgG which bound to and could be eluted from the "helper" T cell column. These antibodies significantly inhibited normal lymphocyte proliferative responses to microbial and histocompatibility antigens. The findings indicate that these SLE plasmas contain immunoglobulins of the IgG class which react with leukemic "helper" T cells and inhibit normal effector T cell function.
Chromosome studies were carried out on 11 National Institutes of Health (N.I.H.) patients who had the diagnosis of Sézary syndrome. Heteroploidy, multiple markers including minute and ring chromosomes, and a lack of modality and clone formation, were the common chromosomal findings in this syndrome. Abundant spontaneous division of heteroploid cells in unstimulated peripheral blood cultures, a high percentage of heteroploid cells in stimulated culture, and finally, clone formation, are signs of a fulminant process and lead to the terminal phase of this disease. Early chemotherapeutic eradication of these heteroploid neoplastic cells would, therefore, be the treatment of choice in this disease.
A comparison of the detailed features and natural history of 12 patients with "Sézary Syndrome" with those in patients with classical "mycosis fungoides" has demonstrated that, with the exception of a leukemic phase in the former, the two syndromes are indistinguishable. Both disorders result from the proliferation of malignant thymus-derived lymphocytes with progressive involvement of skin, lymph nodes, and visceral organs; actual transition between these two syndromes have been documented. "Sézary Syndrome" and "mycosis fungoides" appear to represent variant clinical expressions of the same disease process. It is proposed that the present confusing and archaic terminology should be discarded and that the designation "cutaneous T-cell lymphoma, with or without leukemic phase" should be used in all future descriptions of this disorder.
The present study investigated the effect of vibrotactile stimulation on relaxation as measured by EMG recording. Forty-eight subjects from three age groups were randomly divided into 8 experimental groups: (1) simultaneous footrest vibration and back vibration (A1C1); (2) simultaneous footrest vibration and back roller (A1C2); (3) simultaneous footrest vibration, back vibration and back roller (A1C3); (4) footrest vibration alone (A1C4): (5) back vibration alone (A2C1); (6) back roller alone (A2C2); (7) simultaneous back vibration and back roller (A2C3); and (8) control group (no vibration/stimulation) (A2C4). The three major variables studied were footrest vibration (A1 and A2), pre- and post-EMG measures (B1 and B2), and back vibration (C1C2C3C4). Results showed that footrest vibration had a significant effect on relaxation. Other conditions (except the control) produced a decrease in EMG levels, but did not reach significance. Pre- and postmeasures by experimental conditions were also significantly different. Application of vibration as an aid in relaxation is discussed.
Substantial evidence has accumulated to indicate not only that mycosis fungoides and the Sézary syndrome are closely related malignancies, but to suggest that they are part of a larger spectrum of cutaneous lymphomas. The neoplastic cells of these disorders have membrane features of thymus-derived (T) lymphocytes, a characteristic tissue distribution (skin infiltration, marrow sparing, localization in T-cell regions of lymphoid tissue), and distinctive morphology. For these reasons, we suggest that these lymphoproliferative disorders be grouped together as "cutaneous T-cell lymphomas". The anergy noted in patients of this group with leukemia probably is related to both decreased percentages of normal T cells and presence in the serum of macrophage migration inhibitory activity. Leukapheresis has been particularly effective in the management of selected patients. The homogeneous T-cell populations in the patients with leukemia also provide important opportunities to study many aspects of lymphocyte physiology that are of broad biologic significance.
The abnormal lymphocytes from patients with the Sezary syndrome produce macrophage migration inhibitory factors (MIF) both in vitro and in vivo. Five of six individuals studied had significant serum MIF activity and one had borderline activity. In contrast, in this study 47 normal individuals and 9 of 10 patients with extensive skin disease other than that of the Sezary syndrome had no such activity. Since the neoplastic T cells in patients with the Sezary syndrome localize in the skin, their production of chemical mediators in that site may be responsible for the generalized exfoliative erythroderma seen in that disease.
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Bone-marrow-derived (B) and thymus-derived (T) lymphocytes can be distinguished by the presence of a number of receptors and differentiation antigens. The presence of these markers has facilitated the identification and characterization of the mononuclear cells in a number of animal and human lymphoid malignancies. We describe here the immunological properties of human leukemia cells that are highly unusual, since they simultaneously bear the receptor for sheep erythrocytes characteristic of human T lymphocytes and the receptor for antigen-antibody-complement complexes characteristic of human B lymphocytes. A small number (about 2%) of normal human lymphocytes bearing both of these receptors was also identified.
Over the past two years, the photopheresis treatment program at Yale-New Haven Hospital has treated 32 patients with cutaneous T-cell lymphoma. There were 19 erythrodermic patients who had photopheresis as their first systemic therapy. Five of these cleared 75 percent of their skin and the majority of erythrodermics achieved an improved quality of life. Those with best responses were treated earlier in the course of their disease and had more normal proportions of CD4 and CD8 subsets when compared to patients with poor responses. In addition, patients with tumor stage disease and those patients in relapse after intensive radio- and chemotherapy were treated with photopheresis. These results demonstrate that photopheresis has a role in the management of cutaneous T-cell lymphoma.
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