Résumé
Etanercept is a soluble receptor fusion protein that inhibits tumor necrosis factor-alpha(TNF-alpha). The receptor is used widely in the treatment of ankylosing spondylitis, rheumatoid arthritis, sarcoidosis and other indications. For sarcoidosis, it potentially suppresses granuloma formation with TNF-alpha blocking. On the other hand, recent studies have suggested that paradoxical sarcoidosis can be induced by TNF-alpha antagonists in some cases. A 42-year-old woman, who was treated with etanercept due to ankylosing spondylitis for 5 years, was admitted because of right suprahilar lymphadenopathy on chest radiography. Chest computed tomography revealed an enlargement of supraclavicular, paratracheal, mediastinal lymph nodes. She was diagnosed with sarcoidosis on the supraclavicular lymph node biopsy, which was non-caseating epithelioid cell granuloma and excluded from similar diseases. She was treated for sarcoidosis with prednisolone instead of etanercept.
Sujets)
Adulte , Femelle , Humains , Polyarthrite rhumatoïde , Biopsie , Cellules épithélioïdes , Granulome , Main , Immunoglobuline G , Noeuds lymphatiques , Maladies lymphatiques , Nécrose , Prednisolone , Récepteurs aux facteurs de nécrose tumorale , Sarcoïdose , Pelvispondylite rhumatismale , Thorax , Facteur de nécrose tumorale alpha , ÉtanerceptRésumé
Neurological manifestations as extra-articular features of ankylosing spondylitis (AS) have not been reported often, and present as root lesions, cauda equina syndrome, and compression of the spinal cord. We report a 31-year-old man who complained of back pain and claudication of both lower extremities. The patient showed typical inflammatory back pain and significant sacroiliitis, consistent with AS. He also had mechanical back pain and unexplained neurological symptoms. A neurological evaluation, including spinal magnetic resonance imaging and a cerebrospinal fluid study, revealed that the patient had acute transverse myelitis (ATM). ATM can be associated with connective tissue diseases. The pathophysiology of ATM consists primarily of inflammatory disorders, similar to the neurological complications of AS. We suggest that ATM is a possible extra-articular manifestation of AS.
Sujets)
Adulte , Humains , Dorsalgie , Maladies du tissu conjonctif , Membre inférieur , Imagerie par résonance magnétique , Myélite transverse , Manifestations neurologiques , Polyradiculopathie , Sacro-iliite , Moelle spinale , Pelvispondylite rhumatismaleRésumé
The blast phase in chronic myelogenous leukemia (CML) is associated with mutation of several genes. It is well known that p53 gene mutation plays a key role in the myeloid or lymphoid blast phase of CML. But for the case of the N-ras gene, the association between N-ras mutations and the blast phase of CML is not yet known. We report here on a case of detecting N-ras point mutation without p53 mutation in a 64 year-old man who suffered from the lymphoblastic blast phase of CML.
Sujets)
Humains , Crise blastique , Gènes p53 , Gènes ras , Leucémie myéloïde chronique BCR-ABL positive , Mutation ponctuelle , Stress psychologiqueRésumé
T cell large granular lymphocytic leukemia (T-LGL leukemia) is defined as a clonal proliferative disorder of CD3+ cytotoxic T cells. T-LGL leukemia usually expresses CD3+, CD4-, CD8+, CD16+, CD56- and CD57+ cell markers, and this represents a rearrangement of the T cell receptor (TCR) gene. The clinical course is indolent in most cases, but on rare occasions, when CD3+ and CD56+ are expressed on the leukemic cells, it can be more aggressive. We experienced a patient with T-LGL leukemia and the disease was indolent at the time of diagnosis, and so any specific treatment was not required. Two years after the initial diagnosis, her clinical course became quite aggressive as the CD 56+ cell surface antigen was expressed. We report here on the first case of T-LGL leukemia in Korea and we review the relevant literature.