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PubMed1993

A case of primary localized amyloidosis of urethra.

Amyloidosis of the urethra is a rare disease. The clinical appearance resembles carcinoma of the urethra, so that biopsy is required to make the appropriate diagnosis. Once primary localized amyloidosis of the urethra has been diagnosed, selection of the appropriate treatment for each case becomes important. We report a case of primary localized amyloidosis in the male anterior urethra. This case was treated successfully with urethral dilatation.

Amyloidosis↗
PubMed1993

[A case of systemic primary amyloidosis with BHL and M-protein in pleural effusion].

A 76-year-old man was admitted to our hospital because of productive cough and dyspnea. Chest X-ray revealed cardiomegaly, bilateral pleural effusion, mediastinal lymphadenopathy, and marked bilateral hilar lymphadenopathy (BHL). His symptoms improved after therapy including diuretic for heart failure, but BHL was unchanged. M-protein in both serum and pleural effusion, and B-J protein in urine were detected. Protein immunoelectrophoresis demonstrated a heterogeneous increase in IgA with lambda light chain predominance. Bronchoscopy disclosed some prominent mucosal lesions of the bilateral bronchi, of which biopsies demonstrated amyloid deposition. Gastric mucosal biopsy also demonstrated amyloid deposition. Therefore this case was diagnosed as systemic primary amyloidosis due to AL-protein. In systemic amyloidosis, there are only a few reports of lymph node enlargement due to amyloid deposition, and it is particularly rare with BHL. From the presence of M-protein in pleural effusion, it is suggested that not only congestive heart failure due to cardiac amyloidosis but also amyloid deposition in the pleura would be related to the fluid production.

Aged↗
PubMed1993

Macromolecules that are colocalized with deposits of beta 2-microglobulin in hemodialysis-associated amyloidosis.

BACKGROUND: Common elements in many different types of amyloid may have important roles in amyloidogenesis. The proteinaceous tissue deposits have a common appearance in polarized light and other similar features. The present investigation describes for the first time the relation between beta 2-microglobulin (beta 2-M)-type amyloidosis and colocalized materials, as demonstrated using specific antibodies and hyaluronan-binding protein. EXPERIMENTAL DESIGN: Amyloid-rich carpal tunnel synovium was obtained surgically from 28 patients who were being treated by maintenance hemodialysis. Serial sections were examined using a hyaluronan (hyaluronic acid)-binding protein and antibodies against heparan sulfate-glycosaminoglycan, chondroitin sulfate-proteoglycan, dermatan sulfate-proteoglycan, alpha 1-antichymotrypsin, alpha 1-antitrypsin, inter-alpha-trypsin inhibitor, haptoglobin, and ubiquitin. RESULTS: Accumulation of hyaluronan was of three types, namely, localization around beta 2-M deposits, colocalization with deposition of beta 2-M itself and localization at a small distance from beta 2-M deposits. Immunostaining for heparan sulfate glycosaminoglycan was demonstrated at the sites of beta 2-M plaques. Chondroitin sulfate-proteoglycan did not show specific patterns of immunostaining, resembling hyaluronan rather than heparan sulfate. The other materials tested, alpha 1-antichymotrypsin, alpha 1-antitrypsin, inter-alpha-trypsin, haptoglobin and ubiquitin, were not immunostained at sites of beta 2-M plaques. Sodium dodecyl sulfate-polyacrylamide gel electrophoresis and immunoblotting revealed that the molecular weight of heparan sulfate-glycosaminoglycan was 16,000. CONCLUSIONS: These results suggest that HS has an important role in hemodialysis-associated amyloidosis as it does in other types of amyloidosis. Moreover, accumulation of hyaluronan may be an indication of inflammation of the carpal synovium.

Amyloidosis↗
PubMed1993

[Rapid development of primary amyloidosis after two years' follow-up of monoclonal gammopathy of undetermined significance (MGUS)].

Recently, we have encountered an unusual case of "monoclonal gammopathy of undetermined significance (MGUS)" in which some clinical features indicative of primary and amyloidosis (PA) were observed two years after the diagnosis of MGUS. The patient, born in 1925, was diagnosed of having MGUS (IgG-lambda type M-component level; 1 g/dl) in June 1990. The following clinical features occurred in close succession within five months after a stable course of two years: general malaise, abdominal, distension, pretibial edema and facial puffiness, ecchymoses on the chest wall, and dyspnea on effort. The biopsy specimens from the skin and gastric mucosa revealed amyloid deposition. The M-component levels in the serum and urine as well as the number of bone marrow plasma cells remained unchanged. The various kinds of laboratory examinations indicated that systemic amyloidosis rapidly developed within five months. It seems quite likely that the heart, liver, and spleen may be affected with amyloidosis. It is noteworthy that PA can occur without an increase in the serum M-component level at any time after the diagnosis of MGUS. An early diagnosis of PA as well as multiple myeloma should be kept in mind in the follow-up study of patients with MGUS.

Aged↗
PubMedDate not supplied

Fine needle aspiration of abdominal fat for the diagnosis of amyloidosis.

Amyloidosis is a dysproteinemia characterized by extracellular deposition of amyloid fibrils. Its diagnosis depends on the demonstration of the characteristic apple-green birefringence in tissue stained with Congo red. Aspiration of subcutaneous fat as a means of procuring tissue has received attention in the internal medicine literature. However, this application of fine needle aspiration biopsy has not been investigated extensively by cytopathologists. We therefore report our experience. Over the past two years we performed 18 aspirations of subcutaneous fat on 17 patients in whom amyloidosis was clinically suspected. Eight aspirates were positive for amyloid, and nine were negative. There were no known false-negative results. False positives were more problematic, occurred early in our experience and were due to overinterpretation of occasional long, slender, green strands representing collagen. The true positives were all characterized by large numbers of short, apple-green strands intimately associated with the fat, oriented in multiple directions and occurring in much, if not all, of the aspirated fat. Attention to details of the aspirated material other than the presence or absence of apple-green birefringence can lead to a correct distinction between amyloid and collagen. We conclude that abdominal fat pad aspiration is useful in the workup of suspected amyloidosis, especially since it is a safe, easily performed procedure.

Abdomen↗
PubMed1993

[Obstructive cardiomyopathy, caused by amyloidosis, associated with bone marrow myeloma].

A case of a 40-year-old female is reported. Her symptoms started with abdominal complaints, loss of weight, general weakness, then gradually polyneuropathy, worsening hypotonia and systolic heart murmur developed. The bone marrow showed 20-25% infiltration by myeloma. An IgG lambda type paraproteinaemia was diagnosed using immunelectrophoresis. The ejection systolic murmur was caused by restrictive cardiomyopathy--characteristic of primary amyloidosis--with obstruction of the outflow tract of the left ventricle. Echocardiographic changes took 11 months to develop. The patient's status became dominated by severe hypotonia and she died as a result of heart failure. The post mortem examination confirmed amyloidosis with myeloma. Histopathological examination of the myocardium, coronary and other arteries revealed severe changes with AL-type amyloid deposits. The case demonstrates that amyloidosis can lead not only to restrictive cardiomyopathy but obstruction of the outflow tract of the left ventricle.

Adult↗
PubMed1993

[Disease-modifying factors in chronic polyarthritis. Correlations amongsystemic vasculitis, secondary amyloidosis, septic infections and occurrence of miliary epitheloid-cell granuloma. A review of autopsies].

The following findings were obtained from autopsies performed on 169 deceased with chronic polyarthritis (CP): systemic vasculitis in 26 cases (15.4%), systemic secondary amyloidosis in 32 cases (18.9%), sepsis in 13 cases (7.7%) and miliary epithelioid-cell granulomatosis (probably tuberculosis) in six cases (3.6%). Vasculitis was combined with amyloidosis in five patients, with sepsis in two and with miliary epithelioid-cell granulomatosis in four. Critical random check, using the Mann-Whitney test, did not support significance of relationship between vasculitis and amyloidosis or fatal sepsis, whereas significant correlations were found to exist in CP cases between vasculitis and miliary epithelioid-cell granulomatosis (P < 0.005). The latter had no effect on the severity of vasculitis, but the incidence of the granulomatous type of vasculitis was higher with significance (P < 0.02). The conclusion is that biopsy evidence of granulomatous vasculitis in CP patients should be followed by systematic clinical search for miliary tuberculosis because of above-average incidence of that combination.

Adolescent↗
PubMed1995

Dialysis-related amyloidosis in a large CAPD population.

We studied 212 patients from 13 Italian dialysis centers to evaluate the clinical aspects of dialysis-related amyloidosis in continuous ambulatory peritoneal dialysis (CAPD). The mean age was 64.2 +/- 12.3 years and mean time on dialysis was 36.9 +/- 25.1 months. Residual diuresis was 615.7 +/- 554.0 mL/day and plasma beta 2-microglobulin (beta 2M) level was 27.0 +/- 12.8 mg/L. Radiological skeletal examination, neurological problems related to beta 2M, and urinary and dialytic balance of beta 2M were evaluated. Correlations between age, time on dialysis, residual diuresis, beta 2M plasma levels, beta 2M peritoneal and renal removal, carpal tunnel syndrome, and bone disease were studied. Only the number of bone lesions had a significant positive correlation with patient age and negative correlation with residual diuresis. The latter had an inverse relation with beta 2M plasma levels. Dialytic age did not correlate with any of the parameters. No other correlation was observed. Hand lesions were found in 85% of patients with bone dialysis-related amyloidosis. In conclusion, residual diuresis in our patients played a positive role in the number of bone localizations. Only age, but not time on dialysis, had a positive impact on the bone lesions. The high percentage of hand lesions suggests that the observation of this skeletal segment is a simple, safe, and effective modality of bone follow-up for dialysis-related amyloidosis.

Adult↗
PubMedDate not supplied

[Localized laryngeal amyloidosis. Treatment with CO2 laser].

Amyloidosis of the upper airways is rare. The larynx is the most common site of involvement. Primary amyloidosis may be localized or generalized. We report a case of laryngeal amyloidosis in which amyloid deposits were confined to the larynx (right false vocal cord and subglottis) and successfully treated with CO2 laser.

Amyloidosis↗
PubMedDate not supplied

Diagnostic approach to and follow-up of difficult cases of AL amyloidosis.

BACKGROUND: Routine electrophoretic analysis fails to detect a monoclonal component (MC) in a considerable portion of AL amyloidosis patients. We investigated whether the combination of immunofixation (IF) on agarose gel electrophoresis and bone marrow plasma cell (BMPC) light chain kappa/lambda ratio analysis could contribute to diagnosis in these cases. The possible use of the BMPC kappa/lambda ratio in monitoring the clone was also investigated. METHODS: We performed BMPC kappa/lambda ratio analysis and IF of serum and urine in 16 selected patients with no detectable MC at routine analysis, despite clinical features suggestive of primary amyloidosis. An anti-idiotypic monoclonal antibody specific for the amyloidogenic immunoglobulin and the BMPC kappa/lambda ratio were used to monitor the clone in a patient who underwent autologous peripheral blood stem cell transplantation. RESULTS: Abnormal kappa/lambda ratios were found in 14 (sensitivity 87.5%), and a MC in 12 (sensitivity 75%). Combination of the two analyses confirmed diagnosis in all cases. In one patient changes in the size of the clone, monitored on serial bone marrow aspirates by an anti-idiotypic antibody, paralleled variations of the kappa/lambda ratio. CONCLUSIONS: This study demonstrates that the combined use of IF and the BMPC kappa/lambda ratio is extremely powerful in AL amyloidosis. In addition, the BMPC kappa/lambda ratio should be considered for monitoring the amyloidogenic clone when serum or urine MC is not quantifiable.

Adult↗
PubMed1995

[Primary amyloidosis of bones. MRI aspects].

Primary systemic amyloidosis is a rare cause of vertebralcollapse. We describe a patient who presented multiplecollapsed vertebrae. Magnetic resonance imaging (MRI) demonstrated a diffuse bone marrow abnormality. A diffuse low signal throughout the vertebral bodies on T1 and T2 weighted spin-echo images were observed. A bone biopsy confirmed the suspected diagnosis of vertebral involvementby amyloid. MR imaging can be of interest to evaluate the bone spreading of a known amyloidosis and to help in the diagnosis of amyloidosis in the case of non traumatical vertebral fractures.

Amyloidosis↗
PubMed1977

Amyloidosis developing in experimental nocardia infections.

Swiss white and C57/BL/6J mice inoculated repeatedly with either Nocardia asteroides or Nocardia brasiliensis organisms developed amyloidosis over a 7-month period. Amyloidosis also developed in these mice within 6 weeks following a single large inoculum of either organism, but not in other in-bred mouse strains, suggesting a genetic influence in the pathogenesis of this form of secondary amyloidosis.

Amyloidosis↗
PubMed1996

[Classification of amyloidosis].

Amyloidosis is a collective term for a heterogeneous group of disorders characterized by deposition of a fibrillar, proteinaceous material, amyloid, in various tissues and organs. Increasing knowledge about the different proteins that constitute the amyloid fibrils has made it possible to classify amyloidosis by the fibril protein, which appears more rational than the traditional classification by its clinical symptoms. A group of experts on amyloidosis met in Oslo in 1990 and agreed upon a nomenclature and classification based on the chemical properties of the amyloid fibrils.

Amyloid↗
PubMedDate not supplied

Reduced taste perception in AL amyloidosis. A frequently unnoticed sensory impairment.

BACKGROUND: Sporadic observations suggest a possible taste impairment in AL amyloidosis, but the frequency and intensity of this sensory anomaly are not known. MATERIALS AND METHODS: We submitted 21 AL amyloidotic patients, drawn from subjects referred to the Amyloidosis Study Center of the Department of Internal Medicine and Medical Therapy, University of Pavia, to suprathreshold scaling analysis. RESULTS: Taste acuity was reduced in most of them. Every taste showed independent behavior, and 90% of these patients were hypogeusic. True ageusia for one or two tastes was observed in 35% of patients. No patient was aware of reduced taste acuity. Macroglossia did not seem to play a prominent role in dulling tastes. CONCLUSIONS: Impairment of taste perception suggests that gustative neuropathy is a frequently unnoticed expression of sensory involvement in AL amyloidosis.

Adult↗
PubMed1995

Expression of inflammatory cytokines and adhesion molecules in haemodialysis-associated amyloidosis.

BACKGROUND: The occurrence of various arthropathies including carpal-tunnel syndrome (CTS) in dialysis-associated amyloidosis, a condition caused by the deposition of beta 2 microglobulin (beta 2MG), has been emphasized for several years. We attempted to analyse the pathogenesis of CTS in haemodialysis-associated amyloidosis (HA). METHODS: The expression of cell adhesion molecules and inflammatory cytokines in tenosynovial tissues was determined by using both reverse-transcriptase polymerase chain reaction (RT-PCR) and immunostaining. RESULTS: There was a marked expression of ICAM-1, VCAM-1, E-selectin mRNAs together with increased mRNA expression of inflammatory cytokines (IL-1 beta, TNF alpha, IL-6 and MCP-1) in proliferating synovial tissues. ICAM-1 was expressed not only on vascular endothelial cells, but also on synovial cells. In contrast, both VCAM-1 and E-selectin were exclusively expressed on endothelial cells. Mononuclear cells bearing CD13, CD14, CD33 and HLA-DR with macrophage-like morphology were accumulated in the perivascular area and expressed VLA-4, LFA-1 and Mac-1. Moreover, synovial lining cells, vascular endothelial cells and infiltrated mononuclear cells expressed chemokines such as MCP-1 and MIP-1 alpha. CONCLUSIONS: These data suggest that upregulated expression of inflammatory cytokines and adhesion molecules promotes activation and infiltration of macrophages causing CTS in haemodialysis-associated amyloidosis patients.

Amyloidosis↗
PubMed1996

[Is early diagnosis of amyloidosis AL possible? Consideration on a representative case].

A male patient, 53 year-old, affected by with amyloidosis AL (AAL) with MGUS IgG lambda shows the renal function and the spiro-index yet normal. There is a cardiomyopathy by amyloidosis, but the kinaemia is yet normal. We think to treat the patient with high dose therapy and PBSCs. The appearance of a heart failure and an asystole after the anaesthesia of marrow-harvest precludes this treatment: once more the diagnosis of amyloidosis AL was late. Is the early diagnosis of AAL possible?

Amyloidosis↗
PubMedDate not supplied

[Morphological features of senile and secondary amyloidosis of the iris and sclera in patients with glaucoma].

Morphologic analysis of 173 biopsy specimens of the iris and sclera removed during antiglaucoma surgery in 115 patients with open-angle glaucoma revealed in 44.4% cases extracellular depositions of amyloid, which aggravated the course of glaucomatous process and were pathogenetically significant. Amyloidosis was revealed in the sclera in 82% cases and in the iris in 70%. Amyloid was detected by Congo red staining with control in polarized light and by toluidine blue staining for chondroitin and dermatan sulfates. Vascular and muscular (involving the pupil muscles) forms of amyloidosis were detected in the iris, pericollagen and focal forms in the draining system and sclera. The authors discuss the contribution of amyloidosis to the pathogenesis of glaucoma. These findings confirm M.M. Krasnov's concept about glaucoma polymorphism.

Adult↗
PubMed1996

High levels of circulating beta-amyloid peptide do not cause cerebral beta-amyloidosis in transgenic mice.

We have established transgenic mice that constitutively overproduce the signal sequence and the 99-amino-acid carboxyl-terminal region of the human beta-amyloid precursor protein. The transgenic mice strongly expressed the transgene in multiple tissues under the control of a cytomegalovirus enhancer/chick beta-actin promoter. There were exceptionally high levels of beta-amyloid peptides in the plasma (approximately 17 times or more compared with the human plasma level). Although some transgenic mice from one founder line developed amyloidosis in the intestine, no neuropathology was found in transgenic mice up to age 29 months. Given the absence of cerebral beta-amyloidosis despite extremely high levels of circulating beta-amyloid peptides in the transgenic mice, the results suggest that local cerebral metabolism of beta-amyloid precursor protein may play a predominant role in cerebral beta-amyloidosis in transgenic mice. Such transgenic mice may be useful for the investigation of the etiology of the disease and for the establishment of therapeutic strategies.

Amyloid beta-Peptides↗