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Clinicopathologic findings in dogs with renal amyloidosis: 59 cases (1976-1986).

The medical records of 59 dogs with renal amyloidosis were reviewed. Most dogs with amyloidosis were greater than 6 years old, and females were affected more often than males. Beagles, Collies, and Walker Hounds were at increased risk, whereas German Shepherd Dogs and mixed-breed dogs were at decreased risk. Common historical findings were anorexia, polyuria, polydipsia, lethargy, vomiting, and weight loss. Common laboratory findings were leukocytosis, lymphopenia, nonregenerative anemia, hypercholesterolemia, azotemia, hyperphosphatemia, metabolic acidosis, isosthenuria, cylindruria, and proteinuria. Proteinuria was moderate to severe in most dogs, as assessed by qualitative determination of urine protein concentration, urine protein/urine creatinine ratio, and 24-hour urine protein excretion. Conservative medical management was of little value, and survival ranged from 3 to 20 months in 12 dogs for which this information was available. Moderate to severe diffuse global glomerular amyloidosis was detected in all dogs. Medullary amyloid deposition was multifocal and less severe, but was evident in most dogs. Secondary tubulointerstitial and glomerular lesions were mild or absent in most dogs. Thromboembolism was identified in approximately 14% of affected dogs, underlying inflammatory disease in 37%, and neoplasia in 20%. Laboratory indicators of renal function correlated poorly with histologic lesions, with the exception of glomerular amyloid deposition and "chronic renal disease" index with endogenous creatinine clearance.

Age Factors↗

[Neoplastic development of laryngeal amyloidosis. Apropos of a case].

The authors report the case of a 62 years old patient who presented a squamous laryngeal carcinoma 8 years after the discovery of amyloidosis localized to one vocal cord. In this patient, a relationship would seem to exist between the two pathologies, with degeneration of amyloidosis being responsible, though a random coexistence cannot, of course, be completely excluded. After a review of laryngeal amyloidosis and its management, this particular case is discussed in relation to other cases described in the literature.

Amyloidosis↗

Activities of lysosomal enzymes and levels of serum amyloid A (SAA) in blood plasma of hamsters during casein induction of AA-amyloidosis.

Casein-induced amyloidosis in hamsters was found to be of the AA-type, as shown by SDS-polyacrylamide gel electrophoresis (SDS-PAGE) and amino acid analysis of the major low-molecular weight component of the amyloid fibrils. Levels of serum amyloid A (SAA) and the activities of cathepsin D, beta-N-glucosaminidase, serine esterase, lactate dehydrogenase (LDH) and gamma glutamyl transpeptidase (GGT) were measured in the blood plasma during induction of amyloidosis. During the pre-amyloid phase an increase was observed in all these parameters. During the deposition of amyloid, an increase was observed in the activities of the lysosomal enzymes cathepsin D and beta-N-glucosaminidase, which was significantly correlated with amyloid deposition. Serine esterase activities did not show any relationship to amyloid deposition. LDH and GGT activities were normal in the amyloid phase. SAA levels were lower during amyloid deposition than during the pre-amyloid phase. These findings indicate that a specific release of lysosomal contents from mononuclear phagocytic cells is involved in the pathogenesis of AA-amyloidosis. Amyloid deposition may be the result of: (i) extrusion of intralysosomal protein AA or pre-amyloid, followed by extracellular formation of amyloid fibrils; (ii) secretion of lysosomal enzymes, followed by extracellular cleavage of SAA and subsequent aggregation of protein AA with other components.

Amino Acids↗

Left atrial myxoma associated with systemic AA amyloidosis.

A 76-year-old woman presenting with generalized amyloidosis of the AA-type protein was found to have a left atrial myxoma. Retrospective estimation of the concentration of SAA protein, a serum precursor of AA amyloid, before and after surgical removal of the myxoma, showed that the SAA protein had disappeared after the operation. A common manifestation of myxoma is the development of a severe inflammatory syndrome that sometimes simulates rheumatic fever or bacterial endocarditis. However, to our knowledge, it has never been described in association with amyloidosis. We suggest that atrial myxoma should be added the list of neoplastic and inflammatory diseases predisposing to AA amyloidosis.

Aged↗

Secondary amyloidosis and cystic fibrosis. A morphological and histochemical study of five cases.

The pathological findings of five cases of amyloidosis associated with Cystic Fibrosis are reported. Rectal biopsy led to the diagnosis of amyloidosis in four patients. In three cases the diagnosis was confirmed at autopsy, with involvement of spleen, liver, kidneys, adrenal glands, thyroid and other organs. It seems that Secondary Amyloidosis provokes a significant, although rare, complication of Cystic Fibrosis as greater numbers of these patients survive into adulthood.

Adult↗

[A case of diffuse pulmonary amyloidosis associated with peripheral neurologic signs].

The authors report a case of well-tolerated and prolonged diffuse parenchymal amyloidosis of the lungs associated with signs of peripheral neuropathy. The latest classifications of amyloidosis generally and of bronchopulmonary amyloidosis specifically are reviewed. The problems raised by this particular case, and notably its relation with the neurological signs observed, are discussed.

Aged↗

Ptosis and dermatochalasis as presenting signs in a case of occult primary systemic amyloidosis (AL).

Amyloidosis (AL) is a disease characterized by the extracellular deposition of a complex glycoprotein, part of which is derived from light chains of immunoglobulins. The ocular adnexa can be involved in both systemic primary amyloidosis (AL), usually associated with multiple myeloma or other immunologic disorders, or in a localized form without such systemic implications. We present the case report of a 65-year-old man in whom occult primary systemic amyloidosis (AL), associated with a monoclonal IgG-kappa gammopathy, occurred with presenting signs of ptosis and dermatochalasis secondary to infiltration of the extraocular and orbicularis oculi muscles with amyloid.

Aged↗

[Senile cardiac amyloidosis].

The findings at 1625 autopsies performed by the authors in 45-99-year-olds and literature data have been analysed in relation to clinical and morphological manifestations of senile cardiac amyloidosis (SCA). It occurred in 2.4% of autopsies and accounted for about a half of senile amyloidosis cases recorded by the authors. IAA and SSA are two separate forms of SCA heterogeneous group in which IAA has incidence rate 3 times that of SSA. IAA is a local SCA form, SSA--systemic form (though isolated ventricular affections are possible). The mean age and sex-specific incidence rate differed: 72.3 and 78.2 years for IAA and SSA, respectively; for women IAA incidence is 3.5 times and SSA 2 times that for males. SCA forms presented various amyloidosis degrees, location, spread, as well as amyloid proteins specificity detectable by relevant direct and indirect methods. Differences in clinico-morphological appearance and death causes for IAA and SSA contributed to differential diagnosis between these forms as well as between SSA and PA.

Aged↗

[Characteristics of the aggregative function of thrombocytes in amyloidosis].

In continuity of prior investigations a study was made of platelet aggregation activity stimulated by arachidonic acid in patients with amyloidosis as compared to patients with chronic glomerulonephritis (CGN). Altogether 31 patients with amyloidosis (primary--2, secondary--15, hereditary in periodical disease--14) and 25 CGN patients (latent disease--10, nephrotic--15) were investigated. Considerable changes in platelet aggregation properties in amyloidosis were shown: the absence of aggregation in most of the patients (22) irrespective of a stage of disease and a tendency to prolongation of a latent period in patients in whom it was observed (9). These data were obtained for the first time. Either normal (in patients with latent type) or raised (in patients with nephrotic type) aggregation to a given stimulus was observed in CGN which was well in accord with the results obtained by other authors.

Adolescent↗

[State of humoral and cellular immunity in experimental amyloidosis].

On a model of caseine amyloidosis in 52 rabbits it was shown that in the preamyloid phase the humoral immunity was stimulated, but as soon as the first deposits of amyloid appeared, it was inhibited. The cellular immunity was inhibited starting from the 20th day of the experiment. On the 40th day of the experiment the cellular immunity was found to be less inhibited in the animals with initial deposits of amyloid as compared with the animals with no amyloidosis; on the 60--80th day in progressing of amyloidosis differences in the degree of inhibition of the cellular immunity were obliterated. The degree of fixation of IgG in amyloid was directly dependent on its content in the blood serum and on the "age" of amyloid.

Amyloidosis↗

[Senile amyloidosis].

Basing on the literature data and their own experience the authors have proposed the classification of senile amyloidosis defining senile systemic (cardiovascular) Westermark, senile multi-organic (multisystemic) Schwartz, and senile local (isolated) amyloidosis (isolated atrial, cerebral, aortic, of the pancreatic islets, prostate, and seminal vesicles) with clinicomorphological characterization and data on the prevalence of each type of senile amyloidosis.

Age Factors↗

Primary amyloidosis A. Immunohistochemical and biochemical characterization.

Primary "idiopathic" amyloidosis is usually related to immunoglobulin light chain (AL) associated with immunocytic dyscrasias, while secondary "reactive" amyloidosis (AA) is related to serum amyloid A protein (SAA) and typically occurs with chronic inflammation, malignancy, or familial Mediterranean fever. In the present study, amyloid fibril protein extracted from frozen and paraffin-embedded tissue from a patient (CAR) with primary systemic amyloidosis proved to be AA protein by immunohistochemical, immunochemical, and amino terminal sequence. Extracts from both frozen and formalin-fixed paraffin-embedded kidney and spleen yielded similar monomers and dimers of the AA protein. The additional high-molecular-weight bands and a distinct 12,000-dalton fragment in the amyloid protein extracted from the formalin-fixed paraffin-embedded lung suggest that different processing of proteins, ie, by polymerization and/or degradation, may occur in different organs.

Amyloidosis↗

[Phagocytosis in experimental and hereditary amyloidosis].

The paper is concerned with the results of a study of the phagocytic activity of mouse peritoneal macrophages during induction of casein amyloidosis and neutrophils of patients with hereditary amyloidosis and amyloidosis caused by colchicine. The authors discuss the role of phagocytosis in amyloidogenesis.

Amyloid↗

Primary amyloidosis presenting as a unilateral hilar mass. Report of a case diagnosed by fine needle aspiration biopsy.

A case of unilateral hilar amyloidosis in a 69-year-old man was diagnosed by fine needle aspiration (FNA) biopsy and confirmed by ultrastructural studies. The roentgenographic presentation of amyloidosis solely as a unilateral hilar enlargement is extremely rare, with only one case previously reported in the English literature; this appears to be the first case of hilar amyloidosis diagnosed by FNA biopsy.

Aged↗

[Cardiac amyloidosis. Invasive and noninvasive diagnosis].

Aim of this study is the analysis of clinical, morphologic and haemodynamic features of cardiac amyloidosis. Cardiac amyloidosis was demonstrated histologically in 7 of our patients: in 6 by endomyocardial biopsy during cardiac catheterization, in one at autopsy. The clinical picture was characterized in every patient by signs and symptoms of congestive heart failure. The electrocardiogram showed several non specific signs: low voltage of the QRS complexes, both in peripheral (4/7) and precordial leads (7/7 cases); marked leftward and upward deviation of the QRS axis (6/7 cases); first degree A-V block (5/7); abnormal Q waves (7/7). M-mode and two-dimensional echocardiography invariably demonstrated a typical pattern: a non dilated left ventricle with thickened and hyper-refractile walls, and usually a slight-moderate diffuse hypokinesia. Other common features were a thickening of right ventricular walls, interatrial septum, and atrioventricular and semilunar valves. Computerized analysis of the M-mode tracings disclosed a marked impairment of the indexes of both systolic and diastolic ventricular function in all patients. By correlating electrocardiographic and echocardiographic data, we found in every case a striking disproportion between the low QRS voltage and the high muscle cross-sectional area (an echocardiographic index of left ventricular mass): this pattern appears to be highly suggestive of infiltrative heart disease. Cardiac catheterization (performed in 6 cases) showed an increase of left ventricular (6/6) and right ventricular (5/6) end-diastolic pressure, with a dip plateau pattern in some cases (4/6 of the left, 2/6 in the right ventricle). The cardiac index was decreased in 3/6 cases. Left ventricular angiography confirmed the echocardiographic data of normal volumes and a slight-moderate decrease of the ejection fraction. We conclude that cardiac amyloidosis usually mimics a restrictive cardiomyopathy (severe congestive heart failure with increased ventricular filling pressures, in the absence of severe systolic ventricular dysfunction). This disease can be suspected clinically by the correlation of the clinical, electrocardiographic and echocardiographic data. The final diagnosis requires an endomyocardial biopsy.

Adult↗

[Kidney transplantation in a case of amyloidosis].

Renal transplantation was performed in five patients with various forms of amyloidosis (familial amyloidosis, Mediterranean fever and Crohn's disease). All grafts were functioning one year after the operation. Only one patient died of cardiac shock more than 10 years after transplantation; the other recipients are alive with a functioning graft from 12 to 67 (mean, 41) months after transplantation. No early severe infection was observed in any patient. Graft biopsy obtained in three patients disclosed amyloid deposits in only one: deposits initially (72 months post-transplantation) mild and perivascular, became more prominent in the vessels and extended into the mesangium 4 1/2 years later. Extrarenal amyloid involvement observed in four cases did not lead to serious clinical consequences during the follow-up period. Renal transplantation thus provides an effective treatment of terminal renal failure due to amyloidosis whatever its cause.

Adolescent↗

[Vesical amyloidosis. Apropos of a case and a review of the literature].

A case of primary localized amyloidosis of the bladder in a 60 years old male is described. The initial symptom was hematuria. Cystoscopy revealed a redish angioma-like lesion on the trigone, and the diagnosis was pointed out with biopsy, complementary data, and a close follow-up during two years. In the literature we found 47 cases of primary localized amyloidosis of the bladder and 10 others cases of amyloidosis of the bladder which were part of generalized amyloid disease. The interest of the Wright's special staining method is underlined specifying, on light microscopic study, the AA or AL type of the amyloid disease.

Amyloidosis↗

Primary systemic amyloidosis: comparison of melphalan and prednisone versus placebo.

Satisfactory treatment for primary amyloidosis does not exist. Because the amyloid fibrils consist of a portion of a monoclonal light chain, it appears reasonable to treat amyloidosis with alkylating agents that are effective against the plasma cells that synthesize monoclonal light chains. Fifty-five patients with primary systemic amyloidosis were randomized (double blind) to melphalan-prednisone or placebo. In comparison with the placebo group, patients given melphalan-prednisone were able to continue on treatment for a longer time and to receive larger doses before the code was broken. Among this group, the nephrotic syndrome disappeared in two patients and urinary excretion of protein was reduced by more than 50% in eight others. Of 13 patients who received melphalan-prednisone for more than 12 mo, 6 improved, 3 were stable, and 4 had progression of disease. Survival did not differ significantly between the groups.

Alkaline Phosphatase↗