Cutaneous ␥/␦ T-cell lymphoma (CGD-TCL) is a recent entity described in the newly revised World health organization-European organization for research and treatment of cancer classification of cutaneous lymphomas. Only a few cases have been reported, of which two pediatric cases. A 15 years old child with a 6 months history of polyadenopathy, cutaneous lesions, general edema and deterioration of general condition was hospitalized. Results from laboratory testing, cutaneous histopathology and immunohistochemistry showed a primary CGD-TCL. Staging was completed by a total body computed tomography. Therapy was planified with SMILE protocol. It is a highly aggressive tumor resistant to chemotherapy, immunotherapy, and radiation therapy. The GDTCL is characterized by a worse prognosis with a median survival of 15 months. Early diagnosis is essential and aggressive therapy is necessary.
Aim of the study:The aim was to determine the blood concentrations of hydroxychloroquine in Moroccan systemic lupus erythematosus patients and identify the factors associated with interindividual variation. Methods: A cross-sectional study enrolled patients with lupus erythematosus. We recorded demographic features of patients and main clinical and biological characteristics of the disease. We determined the blood concentrations of hydroxychloroquine for the entire patient enrolled in this study by liquid chromatographymass spectrometry. Patients were divided according to their blood hydroxychloroquine level into three groups: low blood concentration (<500 ng/mL), therapeutic range (500-2000 ng/mL) and high blood HCQ concentration (>2000). Results: Eighty subjects were included; 77 were female and the mean age was 36.9±12, 2 years. The median concentration of hydroxychloroquine was 830 ng/mL (range: 35-3,200 ng/mL); 13 patients (16%) had low blood levels, 59 (74%) were in therapeutic range while 8 (10%) had high blood concentrations. Low body mass index and the use of corticosteroids were associated with high hydroxychloroquine concentrations (p=0.03 and p=0.02 respectively). Conclusion: We report for the first time the blood concentration of hydroxychloroquine in Moroccan lupus patients and we found significant variability between patients for the same dose. Various factors affected this concentration and further studies are needed to expand the sample.
Background The association of inflammatory myopathy and myasthenia gravis is a rarely described entity whose clinical presentation has always been intriguing because of the great clinical similarity between these two pathologies. The presence of a thymic pathology often explains this combination, whose mechanisms are very complex. Case presentation A 56-year-old woman of North African origin, was hospitalized to explore the Raynaud phenomenon associated with proximal muscle weakness, pain, and arthralgia. There was no rash, and neuromuscular examination had revealed proximal tetraparesis and mild neck weakness. Tendon reflexes were normal. There was no abnormal nail fold capillaroscopy. A significant titer of muscle enzymes had been shown on blood tests, and autoimmune screening for myositis-specific and myositis-associated autoantibodies was negative. Electromyography had shown a myopathic pattern, and muscle biopsy confirmed an inflammatory myopathy. Although steroids were introduced, the clinical course was unsatisfactory; ophthalmic and bulbar symptomatology appeared. The association of myasthenia gravis was confirmed by an elevated level of serum acetylcholine receptor. A chest computed tomography scan had identified a thymoma. Treated with prednisone, pyridostigmine, and thymectomy, the patient’s clinical and biological evolution was favorable. Conclusion This case illustrates an exceptional association of two entities and the difficulty encountered during their diagnosis and treatment. The management of these two diseases is different, so it is essential to recognize this concomitant presentation.
Background: Vitamin D plays an important role in the immunomodulation and could be involved in the development of autoimmune diseases such as systemic lupus erythematous (SLE). The study of the polymorphism of the Vitamin D Receptor (VDR) gene may be of interest in explaining the pathophysiology of SLE.Methods: In this study, we aimed to examine the characteristics of VDR gene BsmI polymorphism for the first time in Moroccan patients with SLE and their relationship with clinical manifestations of the disease. We also measured the serum level of 25-hyroxyvitamin D3 to assess its relation to such polymorphism.Results: The study included 66 SLE patients and 91 healthy controls. Our results showed that there were no differences observed in VDR genotypes and allelic distribution within the two groups. Both groups were in Hardy-Weinberg equilibrium, with no significant P values for the observed and expected genotype frequencies. 25-hyroxyvitamin D3 serum levels were the same in the two groups.Conclusions: Based on the results of the present study. We cannot verify any association between VDR gene BsmI polymorphism and SLE. This polymorphism could not be regarded as a genetic marker of the SLE. A larger study examining BsmI and other VDR gene polymorphisms is needed.
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