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Extranodal NK/T-cell lymphoma, nasal type, is a rare and highly aggressive disease with a grim prognosis. No therapeutic strategy is currently identified in relapsing patients. We report the results of a French prospective phase II trial of an L-asparaginase-containing regimen in 19 patients with relapsed or refractory disease treated in 13 centers. Eleven(More)
AIMS Hereditary non-polyposis colorectal cancer is related to germline mutations of DNA mismatch repair genes MLH1 and MSH2, which result in microsatellite instability and loss of protein expression of the corresponding mutated gene in the tumour tissue. METHODS AND RESULTS MLH1 and MSH2 protein expression was studied by immunohistochemistry in(More)
CONTEXT Hyperostosis-hyperphosphatemia syndrome (HHS) is a rare metabolic disorder characterized by hyperphosphatemia and localized hyperostosis. HHS is caused by mutations in GALNT3, which encodes UDP-N-acetyl-alpha-D-galactosamine:polypeptide N- acetylgalactosaminyltransferase 3. Familial tumoral calcinosis (TC), characterized by ectopic calcifications(More)
INTRODUCTION Marginal zone B-cell lymphoma is the most frequent ocular adnexal lymphoma. It may involve the conjunctiva, lacrimal gland, eyelid, extraocular muscle, and orbital connective tissue. Extraocular muscle infiltration is rare. METHODS We report here a series of 5 patients presenting with extraocular muscle infiltration due to marginal zone(More)
BACKGROUND Paragangliomas rarely involve the cauda equina region. In this location, these tumors can show misleading morphological features such as trabecular or papillary growth patterns and variable expression of cytokeratins. METHODS We comparatively studied the immunohistochemical and ultrastructural patterns of 3 paragangliomas of the cauda equina(More)
Biopsies and cell lines of natural killer/T-cell lymphoma, nasal type (NKTCL) were subject to combined gene expression profiling and array-based comparative genomic hybridization analyses. Compared with peripheral T-cell lymphoma, not otherwise specified, NKTCL had greater transcript levels for NK-cell and cytotoxic molecules, especially granzyme H.(More)
BACKGROUND AND OBJECTIVES Small B-cell indolent lymphomas postulated to be of a post-germinal center origin include marginal zone lymphomas of the spleen (S-MZL) or lymph nodes (N-MZL) and mucosa-associated lymphoid tissue (MALT) lymphomas and lymphoplasmacytic lymphomas (LPL). The existence of rather aggressive cases stresses the need for new biological(More)
BACKGROUND Extranodal natural killer (NK)/T-cell lymphoma, nasal type, and aggressive NK-cell leukemia are highly aggressive diseases with a poor outcome. PATIENTS AND METHODS We report a multicentric French retrospective study of 15 patients with relapsed, refractory, or disseminated disease, treated with L-asparaginase-containing regimens in seven(More)