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Atypical Sites (atypical + site)
Selected AbstractsA focal lesion in the falx cerebri: Harbinger of classic stage 4 neuroblastoma in an infant cured despite residual disease after minimal therapyPEDIATRIC BLOOD & CANCER, Issue 7 2009Brian H. Kushner MD Abstract An asymptomatic 11 weeks old received no treatment after he was classified as having a suspected atypical form of MYCN -nonamplified hyperdiploid stage 4S neuroblastoma (NB), with masses in an adrenal gland, subcutaneous tissues, and the falx cerebri. Within 2 months, however, disease progressed in dura and bone marrow. Two cycles of low-dose chemotherapy achieved a partial response; treatment was discontinued. Complete remission was documented 24 weeks post-cycle 2, and has continued >6 years. The falx cerebri probably does not represent an atypical site for stage 4S NB, but stage 4 NB with favorable biology is sometimes curable with minimal therapy. Pediatr Blood Cancer 2009; 53:1340,1342. © 2009 Wiley-Liss, Inc. [source] Genomic organisation and regulation of murine alpha haemoglobin stabilising protein by erythroid Kruppel-like factorBRITISH JOURNAL OF HAEMATOLOGY, Issue 1 2007Janelle R. Keys Summary Alpha haemoglobin stabilising protein (AHSP) binds free , -globin chains and plays an important role in the protection of red cells, particularly during , -thalassaemia. Murine ASHP was discovered as a GATA-1 target gene and human AHSP is directly regulated by GATA-1. More recently, AHSP was rediscovered as a highly erythroid Kruppel-like factor (EKLF) -dependent transcript. We have determined the organisation of the murine AHSP gene and compared it to orthologs. There are two CACC box elements in the proximal promoter. The proximal element is absolutely conserved, but does not bind EKLF as it is not a canonical binding site. In rodents, the distal element contains a 3 bp insertion that disrupts the typical EKLF binding consensus region. Nevertheless, EKLF binds this atypical site by gel mobility shift assay, specifically occupies the AHSP promoter in vivo in a chromatin immunoprecipitation assay, and transactivates AHSP through this CACC site in promoter,reporter assays. Our results suggest EKLF can occupy CACC elements in vivo that are not predictable from the consensus binding site inferred from structural studies. We also propose that absence of AHSP in EKLF-null red cells exacerbates the toxicity of free , -globin chains, which exist because of the defect in , -globin gene activation. [source] The multiform and variable patterns of onset of orofacial granulomatosisJOURNAL OF ORAL PATHOLOGY & MEDICINE, Issue 4 2003Michele D. Mignogna Abstract Background:, The recurrent chronic orofacial swelling caused by orofacial granulomatosis (OFG) can cause significant cosmetic and functional problems but can be prevented if the disease is diagnosed early and promptly treated. Although the enlargement of the lips is described to be the most common presenting complaint, the clinical onset of OFG may be characterized by minor associated mucosal and neurological manifestations, making early diagnosis very difficult or, sometimes, merely presumable. Patients and methods:, We retrospectively analyzed the clinical manifestations of 19 patients with OFG, who were examined at our institution between 1998 and 2002, in order to determine their initial manifestations and presenting symptoms. Results:, A total of 10 patients showed classical recurrent enlargement of the lips (six lower; four upper) as presenting symptom. In the other nine patients, OFG onset was characterized by transient unilateral facial nerve palsy (two cases), intraoral manifestations (two cases), recurrent swelling of the periorbital area (two cases), of the chin (one case), of the zygomatic area (one case), and of the cheeks (one case). Conclusion:, Our data underlined that OFG onset could be frequently characterized by widely variable, multiform, and temporary clinical findings. Involvement of atypical sites of the orofacial region and presence of single minor manifestations may occur as presenting symptoms, often preceding the development of traditional clinical findings. [source] Secretory meningioma of the middle ear: A light microscopic, immunohistochemical and ultrastructural study of one caseNEUROPATHOLOGY, Issue 1 2008Giovanna Cenacchi A 66-year-old woman was referred with left hearing loss. A probable diagnosis of left secretory otitis media with effusion was formulated. A left myringotomy was performed to remove hyperplastic hard tissue from the tympanic cavity. A high resolution CT scan of the temporal bone disclosed a soft-tissue mass completely involving the mastoid and tympanic cavity, surrounding the ossicular chain which appeared spared with no signs of infiltration. The histopathologic, immunohistochemical and ultrastructural response was secretory meningioma, a rare variant of conventional meningothelial meningioma in atypical sites. [source] Nodular lymphocyte predominant Hodgkin lymphoma at atypical locations may be associated with increased numbers of large cells and a diffuse histologic componentAMERICAN JOURNAL OF HEMATOLOGY, Issue 3 2008David T. Yang Nodular lymphocyte predominant Hodgkin lymphoma (NLPHL) typically affects predictable lymph node groups with excellent treatment outcomes, but cases with a diffuse histologic pattern are associated with recurrence and rarely, cases will transform to diffuse large B-cell lymphoma. Although increased numbers of large cells has not been associated with poor prognosis, transformation is thought to histologically progress through a stage distinguished by increasing numbers of large atypical B-cells. From 55 cases of NLPHL, we describe a possible subset of NLPHL occurring in older individuals at atypical sites, associated with increased numbers of large cells, a diffuse histologic component, and expression of Bcl-2. Am. J. Hematol., 2008. © 2007 Wiley-Liss, Inc. [source] | ||||||||||