Showing papers on "Primary systemic amyloidosis published in 1992"
TL;DR: Records of all residents of Olmstead County, Minnesota, with a diagnosis of amyloidosis were obtained from the Mayo Clinic and its affiliated hospitals, as well as other medical groups that might have seen local patients for the period January 1, 1950 to December 31, 1989.
517 citations
TL;DR: The 24-hour urinary protein loss and creatinine values are helpful in predicting which patients eventually will require dialysis and patients whose two-dimensional echocardiograms are normal are most likely to derive long-term benefit from dialysis.
Abstract: . Background. The study assessed dialysis support in pa tients with primary systemic amyloidosis without associated multiple myeloma in whom renal insufficiency developed. Methods. The study group consisted of 211 patients with biopsy-proved primary systemic amyloidosis examined at the Mayo Clinic, Rochester, Minn. No patient was lost to follow-up. Results. Thirty-seven (18%) of the patients received di alysis therapy. Of those presenting with renal amyloid, one third received dialysis. The median time from diagnosis to initiation of dialysis was 13.8 months. The median survival for patients from the start of dialysis was 8.2 months. There was no survival difference between hemodialysis (n=27) and peritoneal dialysis (n=10). The most important predictors of which patients would ultimately require dialysis were the 24-hour urinary protein loss and serum creatinine values at the time amyloidosis was diagnosed. None of the patients seen at diagnosis with a normal serum creatinine value and proteinuria of less than 2 g/d required dialysis during follow up. Of the 37 patients who received dialysis, 31 died, and 21 of the 31 died as a result of extrarenal progression of their systemic amyloidosis. Fifteen of the 31 deaths were a result of cardiac amyloidosis. All long-term survivors had normal echocardiograms without evidence of amyloid. Conclusion. Eighteen percent of patients with primary amyloidosis undergo dialysis. The 24-hour urinary protein loss and creatinine values are helpful in predicting which patients eventually will require dialysis. The median survival for patients starting dialysis is less than 1 year. Patients whose two-dimensional echocardiograms are normal are most likely to derive long-term benefit from dialysis. (Arch Intern Med. 1992;152:2245-2250)
97 citations
TL;DR: Treatment of patients with primary systemic amyloidosis is unsatisfactory and it is reasonable to attempt treatment with alkylating agents that are known to be effective against plasma cell proliferative processes such as multiple myeloma.
Abstract: Primary systemic amyloidosis (AL) is characterized by fibrils composed of the variable portion of a monoclonal light chain. The light-chain class is more frequently A than K (2 : 1). Interestingly, all patients with ILV, subgroup have A],. The incidence of AL is 8.9 per million per year. The median age is 62 years. Weakness, fatigue, and weight loss are the most frequent symptoms. Paresthesias, light-headedness, syncope, change in voice, macroglossia, dyspnoea, paedal oedema, and steatorrhoea may occur [1]. Approximately one-third of patients have a nephrotic syndrome at the time of diagnosis. Congestive heart failure is present initially in about one-quarter of patients, and one-sixth present with orthostatic hypotension or peripheral neuropathy. The presence of one of these syndromes and a monoclonal protein in the serum or urine raises a strong suspicion of amyloidosis. In the Mayo Clinic, 90% of patients with AL have a monoclonal protein in the serum or urine. The diagnosis depends on the demonstration of amyloid deposits in tissue. Tmmunostaining characteristically reveals a reaction of the fibrils with K or i, antisera (see article by Dr Pepys, pp. 519-521). The initial diagnostic procedure should be an abdominal fat aspiration because this test is positive in almost 80% of patients. If it is negative, a bone marrow aspiration and biopsy may be helpful because marrow contains amyloid in about one-half of patients with AL. If the bone marrow and abdominal fat aspirations are negative, a rectal biopsy should be performed. This is reasonably free of complications and produces positive findings in about 80% of patients. If these sites are negative for amyloid, tissue should be obtained from a suspected involved organ such as the kidney, liver, or heart or from sural nerve. The heart is frequently involved. Echocardiography is a valuable technique for the evaluation of amyloid heart disease. Doppler studies are useful and show abnormal relaxation early in the course of the disease, whereas advanced involvement is characterized by restrictive haemodynamics. Treatment of AL is unsatisfactory. The amyloid fibrils consist of the variable portion of monoclonal immunoglobulin light chains that are synthesized by plasma cells. Increased numbers of monoclonal plasma cells are commonly found in the bone marrow of patients with AL. It is therefore reasonable to attempt treatment with alkylating agents that are known to be effective against plasma cell proliferative processes such as multiple myeloma. Colchicine has also been used in the treatment of patients with AL. It inhibits casein induction of amyloidosis in mice and has been effective in the control of abdominal pain and in the prevention of amyloidosis in patients with familial Mediterranean fever. It may inhibit amyloid deposition by blocking the formation of amyloid-enhancing factor. It also inhibits the secretion of amyloid A protein from hepatocytes. It may also interfere with microtubule function. In one study, the median duration of survival was 6 months for 29 untreated patients seen between 1961 and 1973 and 1 7 months for 53 patients seen between 1976 and 1983 who were treated with colchicine [2]. However, for patients in the first group (1961 to 1973), the condition may have been diagnosed later in the course of their disease. Furthermore, supportive therapy may not have been as effective as in the more recent group. In a prospective randomized study of melphalan (0.15 mg/kg per day for 7 days) and prednisone (0.8 mg/kg per day for the same 7 days) every 6 weeks compared with daily colchicine therapy, 101 patients were stratified according to their dominant clinical manifestation. If the disease progressed, the other regimen was added. There was no difference in overall duration of survival when the two groups were compared (the duration was 25.2 months in the melphalan and prednisone group and 18 months in the colchicine group; P = 0.23). When the survival duration of patients who received only one regimen was analysed or when it was analysed from the time of entry into the study to the time of progression of disease or death, significant differences
26 citations
TL;DR: Bowel perforation should be suspected in patients with amyloidosis presenting with acute abdominal pain, and active non-surgical management can be associated with prolonged survival.
Abstract: Spontaneous duodenal perforation in two patients with primary systemic amyloidosis associated with multiple myeloma is described. Bowel perforation is a rare and often fatal complication of amyloidosis, and duodenal perforation has not been previously described. Both patients survived the bowel perforation with conservative management. Bowel perforation should be suspected in patients with amyloidosis presenting with acute abdominal pain, and active non-surgical management can be associated with prolonged survival.
19 citations
TL;DR: Histologically, the whole dermis of the eyelid skin and conjunctiva was replaced by a large quantity of amorphous, eosinophilic substances, which were diffusely positive with Direct Fast Scarlet 4BS.
Abstract: A 52-year-old Japanese woman with the chief complaint of marked swelling of her upper eyelids and a mass over the bulbar conjunctiva is reported. She previously noticed frequent purpura after minimal trauma, which was resolved shortly after taking some ascorbic acid. Laboratory data showed Ig-G kappa type M-protein in the serum and kappa type Bence-Jones protein in the urine by immunoelectrophoresis. Systemic examination showed mild hepatosplenomegaly, 1st degree of AV block, and a mild increase in plasma cells in the bone marrow biopsy. Histologically, the whole dermis of the eyelid skin and conjunctiva was replaced by a large quantity of amorphous, eosinophilic substances, which were diffusely positive with Direct Fast Scarlet 4BS. Immunohistochemical staining was positive for anti-amyloid P component antibody. Typical amyloid fibrils were proved by electron microscopy. She was finally diagnosed as primary systemic amyloidosis with diffuse swollen eyelids and conjunctival mass, symptoms which in primary systemic amyloidosis are very rare.
19 citations
Journal Article•
TL;DR: A case of systemic amyloidosis that has been present in a woman for eighteen years, as manifested by periorbital purpura and an immunoglobulin G kappa light chain paraproteinemia is presented.
Abstract: Systemic amyloidosis can produce a wide variety of clinical manifestations, including characteristic cutaneous findings. Large series of patients with primary systemic amyloidosis have shown that systemic amyloidosis, with or without associated myeloma, has a median survival of no more than twenty-four months. We present a case of systemic amyloidosis that has been present in a woman for eighteen years, as manifested by periorbital purpura and an immunoglobulin G kappa light chain paraproteinemia. She was otherwise healthy; results of bone marrow examination showed no overt myeloma. We speculate that kappa light chain paraproteinemia could prove to be a marker for a more benign type of systemic amyloidosis.
6 citations
Journal Article•
TL;DR: The distinguishing feature of this case was the formation of a solitary and relatively large tumor in the duodenum mimicking a submucosal tumor, which is in contrast to most reported cases of multiple and smaller amyloid tumors in other parts of the gastrointestinal tract.
Abstract: An amyloid tumor of the duodenum is a rare occurrence. A patient who presented with epigastric discomfort, in whom a barium meal study revealed a tumor of the duodenum is described. Upper gastrointestinal endoscopy showed a submucosal location of the tumor, and examination of a biopsy revealed amyloid deposition of the AL type. Diffuse tumor involvement of adjacent tissues of the duodenum and also deposits of amyloid fibrils in the liver and skin with no evidence of a chronic pre- existing disease in the patient led to the diagnosis of primary systemic amyloidosis. The distinguishing feature of this case was the formation of a solitary and relatively large tumor in the duodenum mimicking a submucosal tumor, which is in contrast to most reported cases of multiple and smaller amyloid tumors in other parts of the gastrointestinal tract.
6 citations