23 research outputs found
Unilateral linear basal cell nevus associated with diffuse osteoma cutis, unilateral anodontia, and abnormal bone mineralization
A case of unilateral linear basal cell nevus, diffuse osteoma cutis, unilateral anodontia, and abnormal bone mineralization is reported. Unlike the anodontia, the unilateral linear basal cell nevus and osteoma cutis began to appear in adulthood. A hamartomatous process is suggested to explain these conditions. This complex syndrome has not been reported in the literature
Variable CD7 expression on T cells in the leukemic phase of cutaneous T cell lymphoma (Sézary syndrome).
CD7, a molecule normally expressed on 90% of normal CD4+ T cells, is often deficient on the malignant T cells of cutaneous T cell lymphoma. To investigate the clinical and biologic implications of CD7 expression, blood lymphocytes from 42 patients with the leukemic phase of cutaneous T cell lymphoma (CD4/CD8 ratio of 10 or more with evidence of a T cell clone in the blood) were analyzed for level of expression of CD7 by flow cytometry. CD7 expression by cells did not clearly segregate into two distinct subgroups that are either CD7 positive or CD7 negative as generally thought; however, nine of 17 patients with a predominantly CD4+CD7+ tumor population on early studies became CD4+CD7‒ over time whereas the converse situation was not observed. In addition, of three patients with evidence of large tumor cells in the blood coexisting with smaller cells, discordant CD7 expression was observed in one instance. In lymph node specimens, the percentage of cells expressing CD7 and other T cell markers did not correlate with histologic evidence of involvement. CD7 expression on blood lymphocytes also did not correlate with patients' survival nor to serum IgE levels or blood eosinophil counts, a finding suggesting that this marker does not identify functional cell subsets that produce serum interleukin-4 or -5, respectively. We speculate that the level of CD7 expression on malignant T cells may be the effect of sustained antigen stimulation in vivo analogous to what has been proposed to occur with normal T cells during aging