Biomedical subjects
Publications and source records attributed to W A van Vloten.
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Clinical and laboratory investigations of 8 patients with Sézary's syndrome are presented. Sézary cells were found in peripheral blood as well as in bone marrow and lymph nodes. The overall follow-up period was 8 years. Different therapeutic regimens were given. The best results were obtained with chlorambucil and prednisone orally, combined with topical application of nitrogen mustard. Three patients died of pneumonia and septicaemia.
Red cells of 3 patients with the painful bruising syndrome showed morphological abnormalities. In the fraction not sedimenting in Ficoll/Isopaque gradient centrifugation, some of the cells had club-shaped processes, mitochondria, nuclear remnants, and vacuoles. In freeze-etch preparations, 90% of the red cells showed membrane elevations at pH 7.4 and 25% at pH 6.4, while in freeze-etch preparations of controls these values were 55 and 0 respectively. In addition, rouleaux formation was markedly enhanced in the preparations of blood of patients with the painful bruising syndrome.
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Cell-mediated immune reactivity was studied in 23 patients with mycosis fungoides and Sézary's syndrome by a semi-quantitative 2,4-dinitrochlorobenzene (DNCB) sensibilization test and the reaction to intracutaneous microbial antigens. Anergy to DNCB was observed in 8 patients, 5 had a markedly depressed DNCB score, 6 showed negative reactions to the microbial antigens. A defect in antigen recognition is suggested.
The skin lesions seen in 10 patients who received parenteral nutrition during treatment of chronic enteropathy are described. All of these patients had a lowered serum zinc concentration. The skin lesions were similar to those seen in acrodermatitis enteropathica. After supplementation with zinc sulphate, the skin lesions disappeared completely. A decrease in the serum alkaline phosphatase level can be regarded as a sign of an impending zinc deficiency. Parenteral nutrition formulae should contain a sufficient amount of zinc.
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The ultrastructural and surface marker characteristics of lymphocytes in human cord blood and peripheral blood of healthy donors were studied with respect to the presence of cerebriform mononuclear cells similar to those occurring in the dermal infiltrate of patients with mycosis fungoides (mycosis cells), and the skin infiltrate and peripheral blood of patients with Sézary's syndrome (Sézary cells). Cerebriform monuclear (Sézary-like) cells are characterized by a high nucleus-cytoplasm ratio, deep and narrow nuclear identations, condensed chromatin at the nuclear membrane and cytoplasm poor in organelles. Of the lymphoid cells in human cord blood and peripheral blood of healthy donors 6.7 and 8.7% respectively proved to be cerebriform mononuclear cells. Since these cells invariably form E-rosettes they are part of the T-cell population in healthy individuals. The finding of similar cells in the skin infiltrate of patch test areas of patients allergic to rubber, formalin and peruvian balsam--an expression of cellular immunity mediated by T-cells--suggests that these cells are reactive T cells. Not all (up to 85%) of the cerebriform mononuclear cells in patients with mycosis fungoides and Sézary's syndrome have T-cell membrane characteristics as shown by E-rosette formation. This suggests the presence of two populations of cerebriform mononuclear cells in mycosis fungoides and Sézary's syndrome. The relationship of cerebriform T cells as seen in healthy individuals with cerebriform or atypical mononuclear cells occurring in the Sézary syndrome and mycosis fungoides is discussed.
In 17 patients with DH, multiple duodenal and jejunal biopsies were performed. In all patients the small-intestinal biopsy-specimens showed histopathological changes compatible with coeliac disease. Fourteen of the patients maintained a gluten-free diet (GFD) for more than 8 months. The small-intestinal lesions improved in all patients investigated during the GFD. The dosage of Dapsone needed to control the sin lesions could be reduced by more than 50% in 4 patients and the Dapsone could be stopped in 5 other patients on GFD. The immunoglobulin-bearing cells in the lamina propria were counted in 8 patients not on a gluten-free diet, in 6 patients on gluten-free diet, and in 8 healthy controls. The numbers of IgA-, IgM- and IgG-bearing cells were increased in most of the DH patients who were not on a gluten-free diet. The number of IgM-bearing cells in the DH patients who were on a gluten-free diet was the same as that in the control group. This may indicate a mainly IgM response in the lamina propria induced by gluten.
Total skin irradiation with fast electrons (4 MeV, 3,500 rad) was applied in 10 cases of histologically proven mycosis fungoides without any signs of internal dissemination. The technique is described. Complete disappearance of both lesions and symptoms occurred in all patients after treatment. However, half of the patients relapsed after the initial irradiation and were subsequently treated with a booster dose of 1,000 rad 4 MeV electrons or with topical nitrogen mustard. Irradiation with fast electrons seems to be of great importance in the treatment of mycosis fungoides.
Feulgen-DNA cytophotometry was carried out in skin imprint preparations of patients with mycosis fungoides, reticulum-cell sarcoma, lymphomatoid papulosis and Sézary's syndrome. These patients proved to have aneuploid and polyploid DNA histograms. In tumour stage of mycosis fungoides a more bimodal distribution of the DNA values was found. Of 79 cases of suspected malignant reticulosis of the skin, 32 showed an abnormal DNA histogram with 5 percent or more tetraploid and hypertetraploid cells. Thirty of these patients developed a malignant skin reticulosis during the 4-year follow-up period. It is concluded that DNA cytophotometry has value as a supplement to routine morphological histopathology in malignant cutaneous reticulosis.
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It is well known that in some cases of mycosis fungoides the lymph nodes contain atypical mononuclear cells with a characteristic electron-microscopic morphology, first described in skin lesions of mycosis fungoides. Because it has been shown, that these cells have T-cell membrane characteristics the question can be raised, if these cells have other properties of T cells. One of these is a preferential localization in the T-cell dependent regions (paracortical areas) of the lymph node. In this paper we present a study of dermatopathic lymph nodes from four patients with mycosis fungoides (plaque stage). The lymph nodes of these patients contained atypical mononuclear cells in the paracortical areas only, and not in the follicles or medulla. In one of the patients we could demonstrate the migration of these cells through the epitheloid venules into the paracortical area. Our observations give further evidence of the T-cell nature of the atypical mononuclear cells in mycosis fungoides.
Cytophotometric, cytogenetic, and autoradiographic studies were performed in cells of three patients suffering from clinically diagnosed Sézary's syndrome with erythroderma and the presence of abnormal lymphoid cells in the peripheral blood, skin, bone marrow and lymph-nodes. Feulgen DNA cytophotometry of cells in the peripheral blood and skin lesions showed marked aneuploidy and tetraploidy. Multiple translocations were identified with a G-banding technique. The chromosomal abnormalities varied widely between the patients, but C and D group chromosomes were more frequently involved than others. All breakpoints of the translocations were localised in the centromeric region. Autoradiography of blood and skin samples revealed many labelled cells in the skin and a lower number in the blood, indicating cell proliferation in the skin. It is concluded that the pathological cells occurring in the Sézary syndrome are abnormal lymphoid cells with a tendency to proliferate in the dermis. The variability observed between and in the patients is in all probability due to a difference in the degree of dedifferentiation.
Nine patients with mycosis fungoides in different stages were treated with Bleomycin. Much better results were obtained with this new drug in the six patients with the infiltrative or beginning tumour stage than in the patients in the advanced tumour stage. Complete remission was not seen. In one case the results were objectivized by DNA cytophotometry. The role of Bleomycin in the treatment of mycosis fungoides is discussed. It is concluded that Bleomycin is not the medicament of choice for the treatment of mycosis fungoides.