1/28. Double light-chain disease: a case report.A patient with massive proteinuria was discovered to have double light-chain disease. Immunological studies demonstrated monoclonal light chains of both the lambda and kappa type in urine. The light chains were separate and distinct and were not found to be a part of any of the whole molecule immunoglobulins such as IgG, IgM, IgA, IgD, or IgE. Uniqueness of the proteins was confirmed by column chromatography. Clinical studies showed that the patient had multiple myeloma.- - - - - - - - - - ranking = 1keywords = chromatography (Clic here for more details about this article) |
2/28. pH-dependent fibrillogenesis of a VkappaIII bence jones protein.Disorders of immunoglobulin (Ig) synthesis that occur in malignant plasma-cell proliferation may result in either granular (LCDD) or fibrillar (AL) tissue deposition of light-chain monoclonal components. The structural features that govern the transition from soluble polypeptides to either fibrillar or granular conformational states remain undefined. Among the many factors presumed to play a role in these transitions the net charge of the molecule has been associated with folding conformation changes. The majority of the proteins involved in AL amyloidosis show acidic isoelectric points (pI 3.8-5.2), whereas most L chains with basic pIs deposit in granular patterns. In our studies a 12 kD VkappaIII fragment was purified as the main component of the fibrils isolated from myocardium and adipose tissue of the pericardium obtained post-mortem from an individual with systemic AL amyloidosis. An apparently identical 12 kD VL fragment with the same N-terminal sequence constituted the BJ protein present in the urine. This urinary protein exhibited strikingly cathodic electrophoretic mobility on agarose gels and lacked retention by anionic exchange chromatography matrices, indicative of a highly basic pI (>10). When it was subjected to in vitro fibril-formation experiments, the BJ protein adopted a fibrillar conformation only at acidic pHs, remaining aggregated but not fibrillar at physiological pH. The data indicate that a specific tissue deposition pattern involves not only structural properties of the protein but rather more complex mechanisms in which acidic micro-environments may contribute to the stabilization of amyloidogenic conformations.- - - - - - - - - - ranking = 1keywords = chromatography (Clic here for more details about this article) |
3/28. IgD myeloma. Report of a case with unusual clinical and immunologic features.A case of IgD myeloma in a 48-year-old Caucasian man is reported. The unusual features of this case included the absence of osteolytic lesions by x-ray, absence of anemia, absence of monoclonal spike on serum electrophoresis, association of kappa light chains, absence of Bence Jones proteinemia and Bence Jones proteinuria, and a remarkable, temporary clinical response to therapy. immunoelectrophoresis of whole serum yielded a pattern consistent with IgD kappa monoclonal gammopathy. immunoelectrophoresis of a pure serum IgD preparation, previously separated by gel chromatography (Sephadex G-200), revealed definitive information about an IgD kappa monoclonal gammopathy. Immunomicroscopic examination of bonemarrow smears showed the presence of delta and kappa chains only in the cytoplasm of plasma cells.- - - - - - - - - - ranking = 1keywords = chromatography (Clic here for more details about this article) |
4/28. multiple myeloma presenting as a painful mandibular swelling: a case report.multiple myeloma is a disease characterized by monoclonal proliferation of plasma cells, the most differentiated stage of B-cells. Primary manifestation of multiple myeloma in the jawbones is rare. In the case reported here, a 29-year-old woman who presented with a right mandibular swelling after extraction of a mobile painful tooth turned out to have multiple myeloma. Current diagnostic criteria and management strategies of the disease are discussed.- - - - - - - - - - ranking = 0.0047776141099568keywords = extraction (Clic here for more details about this article) |
5/28. Trimolecular complexes of lambda light chain dimers in serum of a patient with multiple myeloma.BACKGROUND: patients with multiple myeloma often have Bence Jones proteins composed of free monoclonal light chains of the kappa or lambda type in their urine. Usually, these light chains exist as monomeric or dimeric forms, but rarely, larger molecules, such as tetramers, have been reported in the serum. methods AND RESULTS: We report the presence of trimeric complexes of lambda light chain dimers in a patient who was diagnosed with a free lambda light chain multiple myeloma 2 years earlier and subsequently underwent a stem cell transplant. Recently, the patient presented with a large serum M-spike (23 g/L) by protein electrophoresis. The spike consisted of monoclonal lambda light chains without a heavy chain. The urine contained only 8 mg of lambda light chain in a 24-h specimen. Quantitative analysis of the serum and urinary free light chains (FLCs) indicated the probability of larger aggregates of FLCs. Size-exclusion chromatography, electrophoresis, analytical ultracentrifugation, and mass spectrometric studies of the serum revealed almost exclusively the presence of trimolecular aggregates of lambda light chain dimers without other multimeric species. CONCLUSION: Monoclonal lambda light chains may present as hexameric aggregates that cannot be cleared by renal excretion.- - - - - - - - - - ranking = 1keywords = chromatography (Clic here for more details about this article) |
6/28. Myeloma with xanthoderma due to an IgG lambdamonoclonal anti-flavin antibody.When yellow skin and yellow hair developed in an elderly patient with multiple myeloma, we ruled out the usual causes of such pigmentation but identified a monoclonal IgGlambda (lgGGar) with anti-flavin antibody activity. Purified IgGGar was bright yellow, and the acid-dissociated chromophore was identified as riboflavin by chromatography and absorption spectroscopy. Native IgGGar contained 1.45 moles of flavin per mole of IgG, and increased to 2 moles with addition of riboflavin to saturation. The flavin was localized to the Fab fragment and was bound to IgGGar with high affinity. IgGGar showed strongest affinities for riboflavin, flavin mononucleotide and flavin adenine dinucleotide, and lower affinities for dinitrophenyl derivatives and naphthoquinone. The demonstration of hapten bound to the circulating monoclonal immunoglobulin in this case suggests the possibility of bound but colorless haptens on other myeloma proteins as well as on normal immunoglobulins.- - - - - - - - - - ranking = 1keywords = chromatography (Clic here for more details about this article) |
7/28. multiple myeloma presenting as vortex crystalline keratopathy and complicated by endocapsular hematoma.The crystalline keratopathy of multiple myeloma may involve the corneal epithelium, but has not previously been described in a vortex epithelial distribution. Endocapsular hematomas have been described in the period immediately after extracapsular cataract extraction, but not later on or in association with systemic disease. We report a pseudophakic patient who developed a vortex epithelial crystalline keratopathy as a presenting sign of multiple myeloma, and who subsequently developed a spontaneous endocapsular hematoma.- - - - - - - - - - ranking = 0.0047776141099568keywords = extraction (Clic here for more details about this article) |
8/28. Avascular jaw osteonecrosis in association with cancer chemotherapy: series of 10 cases.BACKGROUND: We present a series of 10 patients with osteonecrosis of the jaws (ONJ) that appeared following cancer chemotherapy. MATERIAL AND methods: Of the 10 cases with ONJ, six had bone metastases from breast cancers and the other four had multiple myeloma. We analysed the location of bone metastases, as well as the characteristics of the ONJ, and the drugs with which they had been treated for their bone metastases. RESULTS: Of the 10 patients, all had ONJ in the mandible; 50% also had maxillary involvement. The average number of areas of painful exposed was 2.1 per patient (range 1-5). In seven patients a tooth extraction preceded the onset of ONJ. Two patients developed oroantral communications and another a cutaneous fistula to the neck with suppuration. In all the 10 patients the histopatholological diagnosis was of chronic osteomyelitis without evidence of metastatic disease to the jaws. All the patients had received treatment for their malignant bone disease with bisphosphonates. These were the only drugs that all patients had received. CONCLUSION: ONJ appears to have a relationship with the use of bisphosphonates.- - - - - - - - - - ranking = 0.0047776141099568keywords = extraction (Clic here for more details about this article) |
9/28. jaw bone necrosis without previous dental extractions associated with the use of bisphosphonates (pamidronate and zoledronate): a four-case report.jaw bone necrosis is a clinical condition associated with defects in vascularization of the maxilla or the mandibular bone, usually present following head and neck radiotherapy and/or oral surgical interventions. Bisphosphonates are synthetic analogues of pyrophosphate used in the treatment of patients with hypercalcemia as a result of malignancy, bone metastasis and for the treatment of other disorders such as metabolic bone diseases, Paget's disease and osteoporosis. Over last 10 years, cases of jaw bone necrosis have been associated with the use of bisphosphonate therapy. In particular, Ruggiero et al. (J Oral Maxillofac Surg 2004; 62: 527-534) in 2004 described a large group of patients (63) with jaw bone necrosis probably related to the use of these drugs. It should be noted that all the patients in the group described either underwent head and neck radiotherapy or had a dental extraction while taking bisphosphonates. In the present study, we reported four cases of jawbone necrosis in patients taking pamidronate (Aredia) and zoledronate (Zometa) without having undergone any kind of radiotherapy or dental surgery. All the patients were females between the ages of 56 and 71 years; three were treated with bisphosphonates for bone metastasis and one for multiple myeloma. All the patients received surgical treatment with bone curettage, with partial and/or temporary improvement of the lesions. Although a treatment for bisphosphonate-induced bone lesions has not yet been established, we suggest careful evaluation of the patients' oral health before prescribing bisphosphonate treatment.- - - - - - - - - - ranking = 0.023888070549784keywords = extraction (Clic here for more details about this article) |
10/28. osteonecrosis of the jaws in periodontal patients with a history of bisphosphonates treatment.BACKGROUND/AIM: osteonecrosis of the jaws is being increasingly reported in patients with bone metastasis from a variety of solid tumours and disseminated multiple myeloma receiving intra-venous bisphosphonates. The signs and symptoms that may occur before the appearance of clinical evident osteonecrosis include changes in the health of periodontal tissues, non-healing mucosal ulcers, loose teeth and unexplained soft-tissue infection. A series of nine periodontally involving patients showing osteonecrosis of the jaws that appeared following the intra-venous use of bisphosphonates is reported. MATERIAL AND methods: Nine consecutive patients with osteonecrosis of the jaws were prospectically studied. patients' past medical histories and the drugs that they had received for their malignant disease were systematically documented. Clinical, histopathological and radiographic features and proposal for treatment modalities of osteonecrosis are also reported. RESULTS: Of the nine patients (six women and three men) observed, all had osteonecrosis in the mandible; two had maxillary involvement as well. All nine patients had a history of extraction of periodontally hopeless teeth preceding the onset of osteonecrosis. In two patients, the lesions also appeared in edentulous areas spontaneously. All the patients had received intra-venous bisphosphonates as treatment for their disseminated haematological neoplasms or metastatic bone disease. The duration of bisphosphonate therapy at presentation ranged from 10 to 70 months (median: 33 months). CONCLUSIONS: jaw osteonecrosis appears to be associated with the intra-venous use of bisphosphonates. Dental professionals should be aware of this potentially serious complication in periodontal patients receiving long-term treatment with bisphosphonates.- - - - - - - - - - ranking = 0.0047776141099568keywords = extraction (Clic here for more details about this article) |
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