1/55. A case of amyloid goiter secondary to Crohn's disease.We herewith report a case of amyloid goiter secondary to Crohn's disease. The patient had been diagnosed as having Crohn's disease at the age of 15, and underwent right hemicolectomy at age 20. When he was 26 years old he complained of swelling of the anterior neck. Both TSH and thyroid hormones were within the normal range, and anti-thyroglobulin and anti-microsomal antibodies were negative. Only thyroglobulin was noticeably above the normal range. During the next year his goiter enlarged further and because he had a feeling of pressure he underwent total thyroidectomy. The presence of amyloid A protein in his surgical specimen led to the diagnosis of amyloid goiter. Although most cases of secondary amyloidosis are known to develop in neoplasms or chronic inflammatory diseases, our patient had no illness other than Crohn's disease. Perusal of literature revealed that Crohn's disease is rarely a cause of amyloid goiter.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
2/55. Acute leukemia following prolonged cytotoxic agent therapy.1. Nine patients in whom acute non-lymphoblastic leukemia (ANLL) developed following prolonged alkylating agent therapy are described. Five of the patients received no radiotherapy. The conditions treated were: Hodgkin's disease (four patients), primary amyloidosis, primary macroglobulinemia, malignant lymphoma, multiple myeloma, and carcinoma of the tonsil. 2. Prior to the advent of chemotherapy, this complication was not observed in large series of patients with lymphoproliferative disorders and multiple myeloma. However, the medical literature now contains at least 125 other detailed reports of ANLL developing after prolonged cytotoxic agent therapy. 3. multiple myeloma and Hodgkin's disease, both of which commonly have good responses to chemotherapy, predominate as the underlying diseases. However, 35% of the case reports involve patients with other illnesses, including 12 patients who did not have neoplasms. 4. More than half of the patients developing ANLL have received chemotherapy alone without radiotherapy. 5. At least half of the patients developing ANLL experienced long periods of significant cytopenia during therapy, often with documentation of bone marrow dysplasia. 6. The wide variety of drugs associated with this complication suggests that any cytotoxic agent may be leukemogenic. However, alkylating agents overwhelmingly predominate as the class of compounds which are most often associated with terminal ANLL. 7. The vast majority of patients reported in the literature with ANLL complicating underlying malignancies have received cytotoxic drugs for prolonged periods (median 3 1/2 years) and leukemia developed most commonly 3 to 5 years after the diagnosis of the underlying disease. Most of these patients benefited from therapy and survived longer (median 5 years) than historical control of untreated patients. 8. The leukemogenic potential in man of prolonged cytotoxic agents therapy, especially with alkylating agents, seems to be well established. This evidence admonishes against the prolonged use of these drugs in non-fatal disorders. 9. More accurate assessment of risk: benefit ratios awaits the results of prospective controlled studies. The results of these studies could also lead to significant modifications in recommendations for long-term maintenance therapy with cytotoxic agents.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
3/55. Localized AL amyloidosis of the colon and clinical features of intestinal obstruction. A case report.The term amyloidosis refers to a group of disorders characterized by the extracellular accumulation of insoluble, fibrillar proteins (i.e. amyloid) in various organs and apparatuses. Local deposition of amyloid without systemic involvement is a rather uncommon form that gives rise to an amyloid pseudo-tumor or "amyloidoma". This paper describes the clinical features of an intestinal subocclusion observed in an elderly patient with localized primary amyloidosis of the transverse colon. The endoscopic picture indicated a stenosing neoplasm. Segmentary colectomy, however, followed by histological examination (characteristic green color in polarized light after staining with congo red) and immunohistochemical analysis of the resected tissue, revealed massive deposits of amyloid composed of lambda light chains in the interstitial connective, and perivascular tissue and muscular tunic, and resulted in a diagnosis of AL amyloidosis. This was successfully treated with a combination of melphalan and prednisone.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
4/55. 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 = 0.27045381897782keywords = complex (Clic here for more details about this article) |
5/55. Hypertrophic cardiomyopathy complicated with cardiac amyloidosis.The echocardiographic findings of hypertrophic cardiomyopathy (HCM) are very similar to those of cardiac amyloidosis. A 76-year-old Japanese man was admitted for treatment of early stage gastric cancer in July 1996. His electrocardiogram indicated left ventricular hypertrophy and echocardiography showed left ventricular hypertrophy with asymmetric septal hypertrophy. He was re-admitted complaining of dyspnea on effort and pretibial edema in October 1998. The amplitude of QRS complex on electrocardiogram was decreased. Echocardiogram showed left ventricular wall thickening with granular sparkling. He was diagnosed as HCM with cardiac amyloidosis.- - - - - - - - - - ranking = 0.27045381897782keywords = complex (Clic here for more details about this article) |
6/55. Localized amyloidosis and extramedullary plasmacytoma involving the larynx of a child.Extramedullary plasmacytoma of the larynx and localized laryngeal amyloidosis are 2 uncommon disease entities that are exceedingly rare in children. We report a case of a 12-year-old girl presenting with progressive hoarseness who was subsequently found to have extramedullary plasmacytoma coexisting with localized amyloidosis involving the larynx. Results from the immunohistochemical and molecular studies showed that the tumor cells of the plasmacytoma were monoclonal (kappa-restricted), strongly supporting their neoplastic nature. The biochemical nature of the amyloid deposits was also shown to be of kappa immunoglobulin light chain, suggesting the pathogenetic relationship between the plasmacytoma and amyloid deposition in the larynx of this patient. There was no other evidence of malignancy or amyloidosis elsewhere. On a follow-up period of 4 years, this patient was well and asymptomatic. We believe that this represents the first case in the literature showing the coexistence of extramedullary plasmacytoma and localized amyloidosis of the larynx in children. This case also supports the concept that localized laryngeal amyloidosis may be a manifestation of low-grade B-cell neoplasms.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
7/55. Recombinant human factor viia in the management of amyloid-associated factor x deficiency.factor x deficiency is an important complication of amyloidosis. It is associated with severe bleeding that is difficult to control with plasma or prothrombin complex concentrates. splenectomy ameliorates the factor x deficiency, but achieving satisfactory haemostasis for this operation is problematic. We report that a new clotting concentrate, recombinant factor viia, readily controls bleeding and makes splenectomy feasible.- - - - - - - - - - ranking = 0.27045381897782keywords = complex (Clic here for more details about this article) |
8/55. Amyloid goiter as the initial manifestation of systemic amyloidosis due to familial mediterranean fever with homozygous MEFV mutation.We describe a case of amyloid goiter revealing a systemic amyloidosis secondary to familial mediterranean fever (FMF) with homozygous MEFV mutation, and we review the literature. A 45-year-old euthyroid Sephardic man, known to suffer from FMF, developed a goiter with cold nodule, after which a subtotal thyroidectomy was performed. Histologic evaluation revealed diffuse AA amyloid deposition without any associated thyroid neoplasia. At that time, no other organ was found to be affected by amyloidosis. colchicine and levothyroxine were prescribed. Eight years later, the patient presented with a rapidly growing neck enlargement. He reported that he had discontinued colchicine therapy 2 years earlier. The serum thyrotropin (TSH) and calcitonin levels were normal. Renal, digestive, and salivary gland biopsies confirmed the presence of systemic AA amyloidosis. Despite the reintroduction of colchicine, the onset of compressive symptoms led to the completion of the total thyroidectomy. The histopathology again demonstrated amyloid deposition, and excluded a malignant neoplasm. Nine cases of amyloid goiter associated with FMF have been reported in the literature; none of them had an amyloid goiter as the first manifestation of systemic amyloidosis. To our knowledge, this is the first case of FMF in which an amyloid goiter preceded the development of secondary systemic amyloidosis. The cessation of colchicine therapy may have played a role in local relapse and the secondary spread of amyloid deposits.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
9/55. Extramedullary plasmacytoma of the parotid gland. Report of a case with extensive amyloid deposition masking the cytologic and histopathologic picture.BACKGROUND: Extramedullary plasmacytomas are uncommon. Although approximately 90% occur in the head and neck region, parotid gland localization is extremely rare. CASE: This report describes fine needle aspiration and histopathologic findings in an extramedullary plasmacytoma arising in the left parotid gland of a 62-year-old man. Aspiration smears showed multiple amorphous clumps of material admixed with ductal epithelial cells, multinucleated giant cells and inflammatory cells rich in plasma cells, suggestive of pleomorphic adenoma. In surgical material, excessive amyloid deposition was observed. Six months later the tumor recurred, and in the second surgical specimen clusters of atypical plasma cells among amyloid deposits was noted. Clinical and laboratory examination excluded multiple myeloma. After local recurrence, radiotherapy was applied. Ten months later the patient was well and without systemic involvement. CONCLUSION: Two points are important. First, in the salivary gland region, because of the focal metachromasia of amyloid with Giemsa stain, amyloid can be confused with the chondromyxoid matrix of pleomorphic adenoma. Second, although localized forms of amyloid tumor exist, one should keep in mind that amyloid may be so abundant that it may mask an underlying plasma cell neoplasm, as in our case.- - - - - - - - - - ranking = 1keywords = neoplasm (Clic here for more details about this article) |
10/55. A rare case of dysphagia: hypopharyngeal amyloidosis masquerading as a post-cricoid tumour.Amyloidoses are a group of disorders in which deposition of abnormal amounts of protein complexes (amyloid) occurs in a variety of tissues. The upper aerodigestive tract may be affected, particularly the larynx, but hypopharyngeal involvement is rarely reported. We present a unique case of amyloidosis of the post-cricoid region causing dysphagia. This case report highlights the need for otolaryngologists to consider the possibility of submucosal amyloid deposition, in the absence of mucosal lesions, in patients who present with dysphagia secondary to an obstructive lesion of the post cricoid region.- - - - - - - - - - ranking = 0.27045381897782keywords = complex (Clic here for more details about this article) |
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