Showing papers on "Primary systemic amyloidosis published in 1997"

A Trial of Three Regimens for Primary Amyloidosis: Colchicine Alone, Melphalan and Prednisone, and Melphalan, Prednisone, and Colchicine

Abstract: Background Primary systemic amyloidosis is an uncommon disease characterized by the accumulation in vital organs of a fibrillar protein consisting of monoclonal light chains. Methods We treated 220 patients with biopsy-proved amyloidosis. The patients were randomly assigned to receive colchicine (72 patients), melphalan and prednisone (77), or melphalan, prednisone, and colchicine (71). They were stratified according to their chief clinical manifestations: renal disease (105 patients), cardiac involvement (46), peripheral neuropathy (19), or other (50). Results The median duration of survival after randomization was 8.5 months in the colchicine group, 18 months in the group assigned to melphalan and prednisone, and 17 months in the group assigned to melphalan, prednisone, and colchicine (P Conclusions Therapy with melphalan and prednisone results in objective responses and prolonged survival as compared with colchicine in patients with primary amyloidosis.

Amyloidosis: a review of recent diagnostic and therapeutic developments.

Abstract: Amyloid deposition is associated with a diverse range of disorders that includes Alzheimer's disease, type II diabetes mellitus and dialysis arthropathy. Although less common, systemic AA and AL amyloidosis remain important because effective treatments have increasingly become available. The pathology in all forms of amyloidosis involves the extracellular deposition of protein as characteristic fibrillar aggregates which interfere with tissue structure and function. Amyloid fibrils are derived from different unrelated proteins in the different forms of the disease but share many common properties, including the capacity to bind the normal plasma protein serum amyloid P component (SAP). This is the basis for our development of radiolabelled SAP as a nuclear medicine tracer for the diagnosis and quantitative monitoring of amyloid. Serial studies have shown that the deposits are far from inert but are actually turned over quite rapidly in many patients. The treatment of amyloidosis involves supportive measures whilst every effort is made to reduce the supply of the respective fibril precursor protein. Under favourable circumstances further amyloid deposition will be prevented. existing deposits will regress and improvement of organ function will occur. Since this strategy is not always possible or may fail, new approaches to inhibit fibril formation and promote regression of amyloid are being pursued.

Detection of Epstein-Barr virus in primary cutaneous amyloidosis.

Abstract: Summary To determine the association of Epstein-Barr virus (EBV) with primary cutaneous amyloidosis (PCA), a retrospective study was conducted on skin tissue from 27 Chinese patients with lichen amyloidosus and macular amyloidosis. In situ hybridization with oligonucleotide probes was used to detect the expression of EBV-encoded RNAs (EBERs). Eleven of 27 cases (40.7%) were found to contain the EBV genome. No EBV genome was detected in the skin of the control groups, including three cases of secondary cutaneous amyloidosis, two cases of primary systemic amyloidosis, and four cases of lichen simplex chronicus. Our study showed no correlation between the presence of EBV in PCA patients and the patients'age, sex, clinical type or severity of the skin lesions. Although our results suggest that EBV may be associated with some cases of PCA, the true aetiological role of EBV in PCA remains unknown.

Primary systemic amyloidosis with giant hepatomegaly and a swiftly progressive course.

Abstract: Although the involvement of the liver is common in systemic amyloidosis (AL), clinical features of hepatic dysfunction and liver chemistry abnormalities are often mild or absent. A mild increase in the serum alkaline phosphatase value is the most common finding. Hypertransaminasemia, hyperbilirubinemia, and portal hypertension with ascites and gastroesophageal varices occur late in the course of the disease and predict a short survival. We describe the case of a 58-year-old woman with AL, whose dramatic and unusual clinical picture, consisting of giant hepatomegaly, hypertransaminasemia, increase in alkaline phosphatase, esophageal varices, and ascites, was rapidly complicated by severe obstructive cholestasis.

Primary Systemic Amyloidosis

Abstract: Background: Primary systemic amyloidosis is a rare disease with multiorgan involvement including the skin. Because of the nonspecific and variable presenting symptoms and signs, this condition usually follows a long course before the correct diagnosis is established. Fortunately, skin manifestations contribute greatly in revealing the disease and determining the amyloidosis type. Objective: This article reports a case of primary systemic amyloidosis mimicking nodular primary localized cutaneous amyloidosis of the legs. We describe our systemic findings and the investigation procedure to classify and characterize the amyloidosis type. Methods and Results: After histologic studies revealed amyloid substance in the skin nodules of our 82-year-old patient, she was hospitalized for an appropriate systemic evaluation of this condition and associated medical problems. The investigation included biochemical, immunologic, radiologic, pathologic, and histochemical methods. Conclusion: This case of primary systemic amyloidosis, simulating a localized cutaneous form with nodular lesions, demonstrates the importance of the distinction between both entities. In fact, the management and prognosis will vary significantly.