RÉSUMÉ
<p><b>OBJECTIVE</b>To investigate the pathology, diagnosis and treatment of a patient with hemotidrosis.</p><p><b>METHODS</b>Coagulation tests, coagulation factor activities, von Willebrand factor concentration, bleeding time and platelet aggregation were measured. The bloody exudates from the skin was examined under light microscopy. The involved skin area biopsy was examined histologically.</p><p><b>RESULTS</b>The bloody exudates contained all kinds of normal blood cells mixed with sweat-like fluid, rather than true-sweat. Histopathologic examination showed normal sweat gland structure without blood cells. The patient was successfully treated with propranolol.</p><p><b>CONCLUSION</b>Sympathetic nerve activation in the vasculature might play a role in hemotidrasis, and beta-blockers might be an effective drug for treatment.</p>
Sujet(s)
Humains , Temps de saignement , Tests de coagulation sanguine , Agrégation plaquettaire , Maladies de von Willebrand , Facteur de von WillebrandRÉSUMÉ
<p><b>OBJECTIVE</b>To explore the clinical significance of serum free light chain (sFLC) levels in nonsecretory multiple myeloma (NSMM).</p><p><b>METHODS</b>Nine NSMM patients were hospitalized in our department from Feb 2002 to Sep 2006 and no M-components was found in their serum and urine by immunofixation electrophoresis (IFE). sFLC was assayed by immuno-nephelometry. The clonality of sFLC was estimated by serum kappa:lambda sFLC ratio. Meanwhile, serum immunoglobulin, total kappa and lambda light chain level were also determined in these patients.</p><p><b>RESULTS</b>Increased serum concentrations of either kappa or lambda sFLC (and abnormal kappa/lambda ratios) were detected in 6 of 9 patients with NSMM although their serum immunoglobulin levels were not elevated and total kappa:lambda light chain ratios (1.32 - 2.20) were in the reference range. All the 9 patients had clonal IgH gene rearrangements.</p><p><b>CONCLUSION</b>Quantification of sFLC by immuno-nephelometry is more sensitive than that of serum total light chain measurement and is helpful in estimating the clonality of the light chain in patients with NSMM.</p>
Sujet(s)
Adulte , Femelle , Humains , Mâle , Adulte d'âge moyen , Chaines légères des immunoglobulines , Sang , Myélome multiple , Sang , Néphélométrie et turbidimétrie , Sensibilité et spécificitéRÉSUMÉ
<p><b>OBJECTIVE</b>To analyze the clinical and laboratory features and risk factors of multiple myeloma (MM) with extramedullary disease (EM) and its extraosseous localizations at diagnosis and during the course of MM.</p><p><b>METHODS</b>The clinical features, survival rate and prognostic factors were retrospectively analyzed in 40 patients having EM from a total of 418 MM patients hospitalized in Changzheng Hospital from 1993 to 2006.</p><p><b>RESULTS</b>Among the 40 patients, the first three localizations of EM involved soft tissue, pleura or peritoneum and central nervous system (CNS). Median duration of follow-up was 30 months. The median overall survival (OS) was 28 months. Twenty-five patients (6%) were found to have EM at diagnosis (group A), and their median OS was 16 months and 15 patients (3.6%) developed EM during the course of the disease (group B), and their expected median OS was 72 months. There was a significant difference between group A and B (P = 0.0045) for OS. Compared with those in group A, patients in group B had a higher percentage of plasmacytes (P = 0.022) and plasmablasts (P = 0.029) in bone marrow, and less advanced stage for international staging system (ISS) (P = 0.027). Log-rank univariate analysis showed that higher CRP level, higher serum LDH, Stage II and III for ISS, Hb < 110 g/L at diagnosis were poor prognostic factors. However, multivariate analysis with COX model showed none of them were statistically significant.</p><p><b>CONCLUSION</b>EM tumors are not a rare manifestation of MM. Soft tissue in the commonest area involved. Higher serum CRP and LDH level, more advanced stage for ISS, anemia and having EM are poor prognostic factors of MM.</p>