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PubMed1996

Macular posterior pigmentary incontinence: its relation to macular amyloidosis and notalgia paresthetica.

Patients with clinical features of dorsal macular amyloidosis but without subepidermal amyloid deposits were followed for 2-11 years. The clinical appearance was fairly stable during this period of time, with little tendency of healing. Only 2 of the patients developed typical macular amyloidosis during the follow-up. It is concluded that a condition strongly resembling macular amyloidosis but without amyloid is an entity, and the designation "macular posterior pigmentary incontinence" is proposed. The relationship between macular posterior pigmentary incontinence and the two conditions macular amyloidosis and notalgia paresthetica is discussed.

Adult↗
PubMed1979

Amyloidosis in Malaysian aborigines (Orang Asli).

Material from 334 consecutive autopsies on Orang Asli subjects performed in the University Hospital, Kuala Lumpur between May 1967 and June 1978 was examined for amyloidosis. Nine positive cases were found, all in patients above 40 years of age, giving an age-corrected incidence of about 9%. In 6 cases, amyloidosis was probably secondary to tuberculosis. The remaining 3 cases exhibited a pericollagenous distribution characteristic of primary amyloidosis. Involvement of the heart and lungs was prominent. However, there were considerable similarities in the distribution and staining properties of the amyloid in the 2 groups. Though both the heart and kidney were frequently affected, the kidney was the most common organ to give rise to clinical symptoms. Infection probably plays a major contributory role in amyloidosis in the Orang Asli.

Adult↗
PubMed1977

Amyloidosis and Crohn's disease.

Amyloidosis associated with Crohn's disease was found in 7 patients among 85 subjected to intestinal resection for granulomatous enterocolitis. Most of the patients had symptoms of inflammatory bowel disease of relatively short duration before the diagnosis of amyloidosis was made and were without suppurative complications. Systemic involvement was seen in 6 of the patients. One died postoperatively from renal failure, and in 2 other patients kidney transplantation was performed because of deterioration of a pre-existent renal insufficiency. Six patients were alive 6 months to 10 years after amyloidosis was diagnosed. There is great risk of rapid deterioration of kidney function postoperatively in these patients. However, our experience suggests that in some cases the progression of amyloidosis may be delayed or even brought to a halt after surgical treatment of Crohn's disease.

Adolescent↗
PubMed2000

The analysis of gastrointestinal amyloidosis in 78 patients with chronic renal failure.

Systemic amyloidosis is not a single disease, but the product of a variety of diseases. Amyloid proteins are insoluble fibrils that are deposited extracellularly in many organ tissues. They stain with Congo red and appear apple green under polarized light. Definitive diagnosis and classification ofamyloidosis requires histologic examination of tissue samples. Gastrointestinal tract involvement is common, and all parts of the system can be affected Immunohistochemical studies have shown that amyloid deposited in the gastrointestinal system is most often of the AA, A kappa, or A lambda types. Another type of amyloidprotein, beta-2 microglobulin (beta2M), predominantly affects the musculoskeletal system, and is usually seen in patients who have been on long-term hemodialysis. Mixed systemic amyloidosis (beta2M and AA) is seen only rarely in these patients. In this study, we attempted to answer why this is so, and examined whether or not mixed amyloidosis is related to amyloidogenesis. We studied gastrointestinal tissues from 78 chronic renal failure patients who had systemic amyloidosis with gastrointestinal involvement. A total of 115 endoscopic samples and 1 jejunal resection specimen were analysed immunohistochemically. Immunohistochemical testing using a panel of antisera directed against two major amyloid fibril proteins (AA-Monoclonal, Dako-, and beta2M-Polyclonal, Dako-) showed that all samples contained AA amyloid, but not beta2M type protein. These findings can be explained by the patients' relatively short average duration of hemodialysis and the predominance of endoscopic biopsy samples in our study.

Adolescent↗
PubMed1998

Clinical benefits of diagnosing incipient AA amyloidosis in pediatric rheumatic diseases as estimated from a retrospective study.

OBJECTIVE: The diagnosis of AA amyloidosis could not be made in eight patients with pediatric rheumatic diseases as later verified employing the more sensitive combination of Congo red and additional immunocytochemistry (CRIC). The objective of this paper is to estimate the benefit of CRIC by reevaluating the historical charts with respect to the question as to which of the diagnostic and therapeutic measures would have been altered if the correct diagnosis had been known at the time of the primary biopsy. METHODS: All subsequent biopsies of eight children with historically missed AA amyloidosis in their primary biopsies were retrieved, together with the historical data including the Congo red stains of the biopsies. The biopsies were reexamined blindly for the presence of amyloid and the results were compared with the historical data concerning diagnostic and therapeutic measures. RESULTS: Using CRIC, AA amyloidosis could be identified an average of approximately three years earlier as compared to the historical data. This gain in time would certainly have altered the diagnostic as well as the therapeutic options, i.e. 10 out of 21 biopsies would have been spared and the earlier diagnosis would have initiated more significant antiinflammatory therapy. CONCLUSION: Very early detection of amyloid reduces the diagnostic burden and unveils an option for a consequent antiinflammatory therapy very early in the course of AA amyloidosis.

Amyloidosis↗
PubMed2001

[Aged onset of amyloidosis caused by transthyretin gene mutations].

We have identified that familial amyloid polyneuropathy with middle age onset results from missense mutations of the transthyretin gene. In the present study, we investigated molecular abnormalities of transthyretin in elderly patients with cardiac amyloidosis or amyloid polyneuropathy, using DNA sequencing and protein sequencing. We detected 5 cases of transthyretin-related cardiac amyloidosis using immunohistochemical techniques. All of them had late-onset, mild or no peripheral neuropathy or autonomic dysfunctions, and no family history. Three had transthyretin Met30 and two transthyretin Ile50. We also found 15 patients with transthyretin-related amyloid polyneuropathy. All of them had late-onset and no contributory family history. Twelve had transthyretin Met30, two transthyretin Ile50, and one transthyretin Ser109. Clinical manifestations and sequencing procedures of six representative patients were also presented. Molecular investigation of transthyretin is needed for elderly patients with etiology-unknown cardiac amyloidosis or amyloid polyneuropathy even if there is no family history of amyloidosis.

Aged↗
PubMed2001

A rare cause of unilateral upper and lower eyelid swelling: isolated conjunctival amyloidosis.

Amyloidosis is a term encompassing a group of disorders characterized by the extracellular deposition of a substance called amyloid in various tissues. A very rare cause of chronic ocular discomfort is amyloidosis. We treated a patient with the above complaint in whom both eyelids were diffusely swollen with no other chronic ophthalmic complaints. Transconjunctival biopsy revealed amyloidosis and was confirmed on congo-red staining. Since we are aware that surgical excision may cause eyelid cicatrisation or secondary xerophthalmia, we explained the pros and cons of surgery to the patient. In view of the 12-year history and lack of other symptoms, the patient preferred to be kept under observation. Follow-up at two years did not reveal any significant increase in the size of the swelling. Therefore, we recommend that in a patient with amyloidosis with no visual/ocular impairment, the condition should be left alone without surgical intervention.

Adult↗
PubMed2003

Localized amyloidosis of seminal vesicle and vas deferens: report of two cases.

We reported localized amyloidosis involving seminal vesicles and vasa deferentia, which was found in two patients with prostatic adenocarcinoma. A 60-yr-old (Case 1) and a 59-yr-old (Case 2) man came to our hospital with elevation of serum prostate-specific antigen (PSA) and biopsy proven carcinoma, respectively. MRI revealed multiple irregular foci of low signal intensity in the prostates as well as in both seminal vesicles and vasa deferentia on T2-weighted imaging, suggesting prostatic carcinoma with extension to both seminal vesicles and vasa deferentia in both cases. Under the clinical diagnosis of stage III prostatic adenocarcinoma, a radical prostatectomy was performed in both patients. Microscopically, Gleason score 7 adenocarcinoma was observed in both patients. In addition, isolated amyloidosis of both seminal vesicles and vasa deferentia was found without carcinoma involvement. Localized amyloidosis in the seminal vesicles, which is considered as senile process, has been occasionally reported in the autopsy and in the surgical specimens. Amyloid deposition in the vas deferens has also been reported in the literature, however, the deposition mimicking extension of carcinoma has not been reported. In this report, two cases of isolated amyloidosis of the seminal vesicles and vasa deferentia are described with electron microscopic study and literature review.

Adenocarcinoma↗
PubMedDate not supplied

Primary systemic amyloidosis. Early diagnosis and therapy can improve survival rates and quality of life.

Primary systemic amyloidosis is a rare disease with protean manifestations. Presence of nephrotic syndrome in the absence of diabetes and hypertension, cardiomyopathy in the absence of ischemia, restrictive cardiac defect, demyelinating polyneuropathy, or unexplained hepatomegaly should alert the physician to the possibility of amyloidosis. Initial steps in the diagnostic evaluation of patients with suspected amyloidosis include serum and urine immunoelectrophoresis and immunofixation studies. Demonstration of amyloid material on tissue biopsy (e.g., subcutaneous fat) is required for diagnosis. Availability of effective treatments has improved the outlook of patients with primary systemic amyloidosis. Early diagnosis is critical to optimizing the chances of effective therapy.

Aged↗
PubMed1990

Pulmonary diffuse alveolar septal amyloidosis--diagnosed by transbronchial lung biopsy.

Diffuse alveolar septal involvement is a rare form of pulmonary amyloidosis. Antemortem diagnosis is unusual, and most of the reported cases were diagnosed at autopsy. It has recently been reported that transbronchial lung biopsy via a flexible fiberoptic bronchoscope was a relatively safe method to confirm diffuse alveolar septal amyloidosis. We report a case of pulmonary diffuse alveolar septal amyloidosis confirmed by transbronchial lung biopsy. The patient's chief complaints were dyspnea on exertion and epigastric pain aggravated over a one-year period, while a chest roentgenogram showed bilateral diffuse interstitial infiltration. This case also showed nephrotic syndrome, cardiac arrhythmia, congestive heart failure, a tingling sensation in both hands and multiple nodules in the gastrointestinal tracts, suggesting involvement of the kidney, heart, peripheral nerves and gastrointestinal tracts. We propose that when diffuse interstitial lung disease is present with systemic signs such as nephrotic syndrome or cardiac arrhythmia, amyloidosis should be considered as a possible diagnosis. Also, transbronchial lung biopsy may be a useful confirmative diagnostic tool.

Amyloidosis↗
PubMed2005

[Secondary bladder amyloidosis with severe recurrent hematuria: transurethral Mickuliz procedure as hemostatic option].

OBJECTIVES: Primary localized amyloidosis of the urinary bladder generally has a benign course. On the contrary, secondary amyloidosis, a consequence of systemic amyloidosis, may have massive bleeding and produce complications such as bladder rupture or life-threatening hemodynamic problems requiring desperate hemostatic procedures such as hypogastric artery embolization or ligature, or cystectomy. We report one case in which hemostasis was achieved by a Mickulicz transurethral bladder tamponage. METHODS: 58 year old female with very aggressive rheumatoid arthritis and secondary renal amyloidosis under chronic hemodialysis presenting with severe hematuria after hip replacement. An inflamed bladder was found, the biopsy of which showed edema in all layers with blood vessel walls enlarged by amiloyd deposits. After several unsuccessful transurethral hemostatic procedures, intravesical formalin irrigation was carried out together with a Mikulicz type gauze packaging after urethral dilation. The gauze was withdrawn three days later without bleeding recurrence; however she presented subsequent neurological impairment and finally died 14 days after the last urological procedure. CONCLUSIONS: Transurethral packaging of the urinary bladder in a woman with massive hematuria is a hemostatic option that we recommend to be used before other more dramatic or invasive options are chosen.

Amyloidosis↗
PubMed2005

Resolution of primary amyloidosis by melphalan and prednisolone: a case report.

We here report a case of a 50-year-old man who showed histologically evident resolution of primary amyloidosis by melphalan and prednisolone. The patient was admitted to our hospital for further evaluation of nephrotic syndrome and remarkable hepatomegaly with refractory ascites, on September 11, 1998. Laboratory tests at presentation showed nephrotic syndrome with slight renal impairment and elevation of the enzymes of the biliary system. Monoclonal light chains were not detected in the serum or urine by immunoelectrophoresis. A renal biopsy revealed global deposition of amyloid in all glomeruli, interstitium and blood vessels. Immunofluorescence staining was positive for kappa light chains. Liver biopsy specimens showed extensive deposition of amyloid along sinusoid walls. Bone marrow aspiration contained 7% plasma cells but no clusters or abnormal cells. Based on these findings, systemic AL- (amyloid light chain) amyloidosis was diagnosed, and the treatment with combinations of melphalan and prednisolone was started from October 1998 at intervals of 4-6 weeks. Renal impairment progressed, resulting in the initiation of maintenance hemodialysis in February 1999. Reinfusion of ascitic fluid into the hemodialysis circuit had been performed from March 1999 for refractory ascites, and ascites disappeared in July 1999. Furthermore, urinary output increased after 14 courses of chemotherapy. Renal function gradually ameliorated with a concomitant reduction in the enzymes of biliary system, and finally hemodialysis was discontinued in April 2001. Sixteen courses of chemotherapy were administered by April 2001. Proteinuria was negative in August 2001. A second renal biopsy was performed on November 20, 2001, which showed markedly decreased amyloid deposition and a proliferation of mesangial cells and increase in matrix in various degrees. We report a case of a patient with primary amyloidosis who was successfully treated by melphalan and prednisolone, resulting in marked resolution of renal amyloidosis.

Alkylating Agents↗
PubMed2001

Effect of dose-intensive intravenous melphalan and autologous blood stem-cell transplantation on al amyloidosis-associated renal disease.

BACKGROUND: Dose-intensive intravenous melphalan with autologous blood stem-cell transplantation induces remission of the plasma cell dyscrasia in a substantial proportion of patients with AL amyloidosis. The impact of this treatment on associated renal disease is not known. OBJECTIVE: To determine the effect of dose-intensive intravenous melphalan and autologous blood stem-cell transplantation on AL amyloidosis-associated renal disease. DESIGN: Prospective cohort study. SETTING: Academic medical center. PATIENTS: 65 patients with AL amyloidosis and urinary protein excretion greater than 1 g/24 h who received dose-intensive intravenous melphalan and autologous blood stem-cell transplantation between 1 July 1994 and 30 June 1998. MEASUREMENTS: 24-hour urinary protein excretion, serum cholesterol level, serum albumin level, creatinine clearance, urine and serum immunoelectrophoresis, and bone marrow biopsy. Renal response was defined as a greater than 50% reduction in urinary protein excretion in the absence of a 25% or greater reduction in creatinine clearance. Complete hematologic response was defined as absence of detectable monoclonal protein in serum and urine and a bone marrow specimen containing less than 5% plasma cells without clonal dominance of kappa or lambda isotype. RESULTS: Among the 50 patients who survived for at least 12 months, proteinuria, hypoalbuminemia, and hypercholesterolemia improved during follow-up; 36% met criteria for a renal response. Median 24-hour urinary protein excretion decreased from a baseline value of 9.6 g/24 h to 1.6 g/24 h at 12 months among patients with complete hematologic response, and 71% met criteria for a renal response. Twenty-hour urinary protein excretion did not decrease during follow-up among patients with persistent plasma cell disease, and only 11% had a renal response at 12 months (P < 0.001 for hematologic responders vs. nonresponders). CONCLUSION: Dose-intensive intravenous melphalan with autologous blood stem-cell transplantation improves the nephrotic syndrome in patients with AL amyloidosis-associated renal disease. The benefit is largely limited to patients achieving eradication of the underlying plasma cell dyscrasia.

Adult↗
PubMed2004

High-dose melphalan and autologous stem-cell transplantation in patients with AL amyloidosis: an 8-year study.

BACKGROUND: AL amyloidosis is a fatal disease resulting from tissue deposition of amyloid fibrils derived from monoclonal immunoglobulin light chains. Treatment with oral chemotherapy is minimally effective. OBJECTIVE: To test survival and organ response in a large sample of patients treated with high-dose intravenous melphalan (100 to 200 mg/m2) and autologous blood stem-cell transplantation. DESIGN: 8-year longitudinal analysis of clinical effectiveness. SETTING: University-affiliated specialty referral clinic. PATIENTS: 701 consecutive new patients with AL amyloidosis. INTERVENTION: High-dose chemotherapy and autologous stem-cell transplantation for patients who met eligibility requirements based on organ involvement and clinical status. MEASUREMENTS: Survival analysis of all patients evaluated and a detailed analysis of treatment outcome in the subgroup that received high-dose melphalan and stem-cell transplantation. RESULTS: Among 701 patients with AL amyloidosis, 394 (56%) were eligible for high-dose melphalan and stem-cell transplantation; 82 did not proceed with treatment because of patient choice or disease progression. Median survival of the 312 patients who initiated treatment was 4.6 years. A complete hematologic response, defined as no evidence of an underlying plasma cell dyscrasia 1 year after treatment, was achieved in 40% of patients and was associated with prolonged survival. Statistically significant improvements occurred in end-organ disease and were greater in patients with a complete hematologic response. Mortality rate within 100 days of treatment with high-dose melphalan and stem-cell transplantation was 13%; patients with cardiomyopathy had the highest mortality rates. CONCLUSIONS: Treatment of selected patients with AL amyloidosis by using high-dose melphalan and stem-cell transplantation resulted in hematologic remission, improved 5-year survival, and reversal of amyloid-related disease in a substantial proportion.

Aged↗
PubMed1979

Amyloidosis in chronic heroin addicts with the nephrotic syndrome.

Four chronic heroin addicts had renal amyloidosis and the nephrotic syndrome. Amyloidosis had the secondary type of tissue distribution and developed after many years of addiction. That chronic immunologic stimulation by one or more exogenous antigens or multiple acute inflammatory episodes were important factors in the pathogenesis of amyloidosis in these patients is postulated. Amyloidosis, although uncommon, must be considered among the different types of renal involvement that are known to occur in drug addicts, especially those with the nephrotic syndrome, and amyloid should be specifically searched for in their renal biopsies.

Adult↗
PubMed1996

Isolated amyloidosis of the colon.

A 46-year-old man was seen with a 2-month history of crampy abdominal pain and recent onset of hematochezia. Colonoscopic examination revealed a semiannular mass lesion in the descending colon which was thought to represent a near-obstructing neoplasm. A partial colon resection was performed. Gross inspection revealed a segment of bowel with focally necrotic mucosa but no mass lesion. Microscopic examination revealed deposits of amyloid infiltrating the muscularis propria, submucosal vessel walls, and lamina propria, with focal ischemic necrosis of mucosa. Special stains were positive for light chains, indicating primary amyloidosis. Follow-up studies for multiple myeloma and inflammatory disorders gave negative results. A diagnosis of isolated amyloidosis of the colon was rendered. Two months after surgery, the patient had a recurrence of symptoms and colon biopsy specimens revealed amyloidosis. The patient was given colchicine, with subjective and objective improvement. The various types of amyloidosis are discussed.

Abdominal Pain↗
PubMed1976

Geographical distribution of amyloidosis in Papua New Guinea.

During an 11 year period 73 cases of amyloidosis were diagnosed at Port Moresby General Hospital as a result of histological examination of autopsy or biopsy material. No case of amyloidosis came from either the Highlands or North Coast regions although these two areas together accounted for 16% of the medical admissions at the hospital and made up a similar percentage of Port Moresby's Papua New Guinean population. Within the sub-district in which Port Moresby is situated there was a much higher incidence of amyloidosis in the inland, more sparsely populated, census divisions of the sub-district. Aetiological factors which may be related to the common occurrence of amyloidosis in Papua New Guinea are discussed in light of the observed geographical distribution of cases.

Amyloidosis↗
PubMed1999

[Urokinase as a blood and urine plasminogen activator in chronic glomerulonephritis and amyloidosis].

To estimate the individual role of the plasminogen activators (PA) urokinase (u-PA) and tissue (t-PA) in the development of two renal diseases (the nephrotic forms of chronic glomerulonephritis (CGN) and amyloidosis, the baseline plasma and urine levels of u-PA and t-PA antigens, their functional activity (FPAA), and changes in these parameters were determined after protein loading test (0.7 g/kg). In healthy individuals and patients with amyloidosis, the baseline FPAA changes from 0 to the maximum were caused only by the alterations of u-PA levels, in those with CGN, they were induced by the changes in the content of u-PA and t-AP antigens. The functional loading test revealed PA reserves solely in patients having a high baseline FPAA for both nephropathies: u-PA in amyloidosis and t-PA in CGN. In all the patients, the urine levels of u-PA antigens were 20-40 times more than those of t-PA antigens and 5-6 times less than those plasma u-PA. The findings suggest that urokinase may be regarded as the major plasminogen activator involved in CGN and amyloidosis.

Amyloidosis↗