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Objective To analyze the clinicopathologic features of primary cutaneous CD30 positive lymphoproliferative disorders. Methods A clinical, pathological and immunohistochemical analysis was carried out in 4 cases of lymphomatoid papulosis and 5 cases of primary cutaneous anaplastic large cell lymphoma. Results Lymphomatoid papulosis was divided into 3 subtypes, A, B and C. The lymphomatoid papulosis of subtype A was pathologically characterized by pleomorphic anaplastic large cells or Steinberg-reed cells scattered or patchly distributed among many inflammatory cells; subtype B showed pathological changes characteristic of granuloma fungoides, and manifested as a broad infiltration zone of lymphocytes in dermis with scattered small- to middle-sized atypical gyrus-like lymphocytes; subtype C was characterized by a diffuse distribution of anaplastic large cells and could clinically subside spontaneously. Primary cutaneous anaplastic large cell lymphoma clinically manifested as subcutaneous nodules or papules, and was pathologically characterized by large, pleomorphic, round or ellipse cells with plentiful, eosinophilic or bicolor cytoplasm, large nuclei and obvious nucleoli. The neoplastic cells characteristically expressed CD30 antigen in both lymphomatoid papulosis and primary cutaneous anaplastic large cell lymphoma, and all the cases showed a favorable prognosis.Conclusions Primary cutaneous CD30 positive lymphoproliferative disorders are a spectrum of cutaneous T cell lymphoma with favorable prognosis, and a synthetic analysis of clinical, histopathological and immunohistochemical findings is beneficial to the diagnosis of these entities.
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Objective To report one case with congenital ichthyosiform eruption, neurosensory deafness and vascularizing keratitis. Methods The overall clinical and laboratory examinations were conducted to confirm the diagnosis of keratitis, ichthyosis and deafness (KID) syndrome. Results The case presented with the typical hypotrichosis features of the eye lashes and eyebrows, alopecia of the scalp, and ophtalmological lesions. The keratotic plaques over the face, nose, ears, and the extremities were characterstic, and the skin of the trunk was leather-like, dry and hyperkeratotic. Dysplasia of cerebellum, and cystic enlargement of the fourth ventricle of cerebrum, and Dandy Walker syndrome were observed on MRI scanning. Treatment with oral acitretin for 3 weeks cleared the hyperkeratotic ichthyotic lesions on her posterior scalp and also improved other lesions on the extremities and the trunk. Conclusion Acitretin seems to be promising in the treatment of keratotic skin lesions in KID syndrome.
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Objective To investigate whether systemic lupus erythematosus (SLE) T cell function disorder is related to abnormal biochemical pathways.Methods After cross linking of anti CD3 mAbs to sheep anti mouse IgG and stimulating T cells,the changes of free calcium ion within T cells and these changes under interference of Thapsigargin and EGTA were observed respectively for 10 minutes with an adhesion cytometry.The relation between [Ca 2+ ]i response in SLE T cells and expression of CD3 molecules,or InsP 3 levels was evaluated.Results The base [Ca 2+ ]i response in T cells of SLE patients was similar to that of normal control ( P =0 105).Peak and plateau [Ca 2+ ]i responses were significantly higher in the group of SLE patients ( P
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Objective To demonstrate that SLE T cells primary function disordor was related to abnormal [Ca 2+ ]i responses,and to investigate the reason of abnormal [Ca 2+ ]i responses.Methods After cross-linking of anti-CD 3 mAb to sheep anti-mouse IgG and stimulating T cells,the changes of free calciumion [Ca 2+ ]i within T cells under interference of Thapsigarain and EGTA was observed respectively successively for 10 minutes by an adhesion cytometry.The relation between [Ca 2+ ]i responses in SLE T cells and InsP 3 levels was evaluated .Results The basic [Ca 2+ ]i response in T cells from SLE patients was similar to that from normal control(P=0 105),peak and plateau [Ca 2+ ]i responses were significantly higher in the group of SLE patients(P
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Objective To determine the degree of C 4 changes at precede or coincide with changes in systemic lupus erythematosus (SLE) activity by 5 global activity indices(PGA, M-SLEDAI, M-LAI, SLAM, and M-BILAG), and to evaluate the correlation between changes in C 4 levels and SLE activity in individual organ systems.Methods 53 lupus patients were observed monthly for 1 year in a longitudinal study. Lupus disease activity rate and complement levels were measured at each visit. Disease activity rates were calculated for subgroups defined by previous or concurrent changes in C 4 levels. Logistic regression models were used to determine the significance of the correlation between recent changes in complement levels and disease activity, and between changes in C 4 levels and SLE activity increased in specific organ systems.Results Lupus disease activity occurred at 12%,25%,13% and 12% respectively on PGA,M-SLEDAI,M-LAI,SLAM and M-BILAG. Disease activity by the M-LAI were more frequent when there was a concurrent decrease in C 4. Higher disease activity rates by the SLAM were correlated with previous increases in C 4. Decreases in C 4 were correlated with a concurrent increase in renal disease activity,or related to a concurrent decrease in the hematocrit levels and platelet count.Conclusions Reducing serum in C 4 levels were not consistently correlated with SLE disease activity, decrease in C 4 was correlated with a concurrent increases in renal and hematologic SLE activity.