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PubMedDate not supplied

Fine needle aspiration diagnosis of orbital plasmacytoma with amyloidosis. A case report.

Fine needle aspiration (FNA) cytology of the orbit was used to diagnose an unusual case of multiple myeloma with secondary amyloid deposition in a 27-year-old man who presented with bilateral keratoconjunctivitis and left-sided ptosis. The FNA smears revealed immature plasma cells, extracellular eosinophilic material (amyloid) and numerous multinucleate giant cells. Subsequent histologic studies of excised tissue confirmed the cytologic diagnosis of plasmacytoma with secondary amyloidosis. Amyloid was further confirmed by electron microscopy. The patient was subsequently diagnosed as having multiple myeloma. The cytologic features of amyloidosis and plasmacytoma are presented. The differential diagnosis and the diagnostic utility of FNA cytology in the evaluation of these orbital lesions are also discussed. This apparently was the first case of orbital amyloidosis diagnosed by FNA cytology.

Adult↗
PubMed1994

[Adrenal insufficiency in patients with kidney failure secondary to amyloidosis. Its detection by a stimulation test].

In order to know the prevalence rate of adrenal insufficiency among patients with renal failure due to amyloidosis; we have performed a stimulation test with synthetic corticotropin hormone (Nuvacthen test) in nine patients diagnosed of renal amyloidosis and in five controls. We also measured ACTH and plasma cortisol levels. In two out of nine patients (22%) a low ACTH, low plasmatic cortisol and an abnormal response to Nuvacthen test were detected being diagnosed of primary adrenal insufficiency. We conclude that adrenal insufficiency is not an infrequent finding in systemic amyloidosis and may be due to secondary amyloidotic infiltration of adrenal or pituitary glands. Though Nuvacthen test is a useful test to diagnose subclinical adrenal insufficiency, in our series patients with an abnormal response also had low levels of basal cortisol and ACTH and no one patient with normal basal levels had an abnormal response to the stimulation test.

Adrenal Insufficiency↗
PubMed1994

[Bladder amyloidosis in a female patient with suspected bladder carcinoma].

We report on a patient with a long history of rheumatoid arthritis who consulted for intense hematuria that was initially ascribed to carcinoma of the bladder. Subsequent patient evaluation, however, disclosed amyloidosis of the urinary bladder. The literature on amyloidosis of the urinary tract is reviewed. A detailed analysis of the biopsy specimens taken from a lesion that looks like a bladder carcinoma may disclose other pathologies such as amyloidosis of the urinary bladder.

Aged↗
PubMed1994

Localized amyloidosis of the nasopharynx.

A case of localized amyloidosis of the nasopharynx is presented. This is only the seventh case report of nasopharyngeal amyloidosis to the best of our knowledge. Though rare, this disease should be diagnosed early and properly treated with surgery. All patients of localized disease should be followed up for any evidence of systemic amyloidosis.

Aged↗
PubMed1994

[Esophageal amyloidosis secondary to rheumatoid arthritis simulating achalasia].

The involvement of the esophagus in amyloidosis secondary to rheumatoid arthritis is rare. The case of a female patient with rheumatoid arthritis and secondary esophageal amyloidosis (type AA) with a radiologic and endoscopic clinical picture compatible with achalasia is presented. In the manometry carried out after two cardiomyotomies, abundant non propulsant tertiary waves were seen, as were two primary waves, intraesophageal pressure higher than that of the gastric fundus and lower hypertensive esophageal sphincter which was completely relaxed on one occasion, resulting in a manometric pattern which was different to that of the other two cases published with the same disease. Endoscopic cardiomyotomies and dilatations were performed. The patient died after 2 years due to post dilatation esophageal perforation. The manometric findings are compared with those of the two previous similar cases with emphasis being made in that the anarchic arrangement of the amyloid in the esophagus produces different unforeseen and uncharacteristic motility patterns. The authors only found in the literature two cases of esophageal amyloidosis secondary to rheumatoid arthritis simulating achalasia.

Amyloidosis↗
PubMed1975

Comparison of lung cancer and amyloidosis in rabbits induced by chemical carcinogens.

For the purpose of inducing experimental lung cancer, carcinogens were applied into the bronchus of the rabbit. Experiments were divided into 2 groups, I and II, depending on the methods of application of chemical carcinogens into the bronchus. In Experiment I, carcinogens (4-nitroquinoline 1-oxide and/or 3-methylcholanthrene) in rabbit plasma or distilled water were instilled in the lower bronchus and, in Experiment II, 3-methylcholanthrene in Tween 60 was swabbed on the maon bronchus through a specially made bronchoscope. (1) In Experiment I, 163 of 366 rabbits having received over 4 instillations of the chemical carcinogens and surviving for more than 30 days developed lung cancer. However, in Experiment II, only 2 of 65 rabbits surviving more than 60 days developed lung cancer. (2) In Experiment II, 39 (60%) of 65 rabbits were found to have developed amyloidosis. However, in Experiment I, 86 (approx. 23%) of 366 rabbits developed amyloidosis. Amyloidosis was observed to occur in the kidneys, spleen, liver, and adrenals in both experiments.

4-Nitroquinoline-1-oxide↗
PubMedDate not supplied

High-dose therapy and autologous transplantation in amyloidosis-AL.

A 53-yr.-old woman with amyloidosis AL was treated with high-dose chemotherapy and autologous stem cell infusion in an attempt to suppress the amyloid secretion. A diagnosis of MGUS had been made six years earlier. During the last year her disease had progressively shifted to a full-blown picture of amyloidosis AL, with renal failure, proteinuria, renal amyloid deposition and plasma cell sheets in the marrow. After an unsuccessful attempt with standard-dose chemotherapy, she received a high-dose regimen of busulphan (14 mg/Kg) and melphalan (40 mg/m2), followed by the infusion of both autologous bone marrow and peripheral blood stem cells. She had full and prompt engraftment, but eight weeks post-graft developed interstitial pneumonitis: CMV was isolated. The patient died while in the intensive care unit. In the literature, this is the first case of amyloidosis AL treated with high-dose therapy and autologous transplantation.

Amyloidosis↗
PubMed1993

Cardiac amyloidosis presenting as sick sinus syndrome and intractable heart failure: report of a case.

Cardiac amyloidosis, an uncommon disease, has been reported to manifest as congestive heart failure (CHF) and/or various arrhythmias. Herein, we report a case of CHF and sick sinus syndrome. The patient, a 66-year-old man, was admitted to the National Taiwan University Hospital because of dizzy spells and recurrent syncope. Electrocardiogram showed a sinoatrial block, first degree atrioventricular block, right bundle branch block and low-voltage Q wave, R wave and S wave (QRS) complex. Prolonged corrected sinus node recovery time was documented by an atrial pacing study. A permanent pacemaker was implanted for the patient's bradyarrhythmia, but he developed progressive heart failure. Echocardiography revealed a normal-sized ventricular chamber, concentric left ventricular hypertrophy with a "granular sparkling" appearance of the myocardium, and impaired diastolic and systolic function of the left ventricle. Despite aggressive treatment, the patient expired due to intractable heart failure. Postmortem needle aspiration revealed amyloidosis involving the heart, lung and skin. We conclude that cardiac amyloidosis should be considered in elderly patients with conduction disturbance and unexplained congestive heart failure.

Aged↗
PubMed1993

[Hypernephroma and associated AL-amyloidosis with polyneuropathy in monoclonal gammopathy].

Malignant neoplasms may have secondary (reactive, AA), amyloidosis as a sequel. Among carcinomas, this is most frequent with hypernephroma. We report a 60-year-old male patient with progressive polyneuropathy and monoclonal gammopathy of the kappa type. A renal tumor was histologically diagnosed as hypernephroma at nephrectomy. The patient did not improve postoperatively and died three and a half months later from pulmonary embolism. Autopsy revealed a systemic amyloidosis, predominantly with prominent deposits in peripheral nerves. Immunohistochemical staining demonstrated the amyloidosis as of the primary (AL) type, rather than of the expected AA type. A plasmocytoma was not detected, either clinically or at autopsy. We assume here a coincidental rather than causal connection between the hypernephroma and the monoclonal gammopathy with AL amyloid and the neuropathy.

Amyloid↗
PubMed1994

[Restrictive infiltrating cardiomyopathy in primary amyloidosis].

The authors describe a typical echocardiographic finding in restrictive infiltrative cardiomyopathy which after two years led to the diagnosis of primary amyloidosis in a 51-year-old woman. The predominating manifestations of the disease were renal damage with final failure, heart failure, monoclonal gammapathy and damage of other organs. Aimed treatment of amyloidosis and repeated haemodialysis did not prevent the patient's death from cardiac and renal failure. Primary amyloidosis was confirmed by histological examination and necropsy.

Amyloidosis↗
PubMedDate not supplied

The association between Henoch-Schönlein syndrome and renal amyloidosis: a proposal of a pathogenic mechanism.

The clinicopathological analysis of 250 pediatric cases that had been tissue-diagnosed with renal amyloidosis revealed three patients associated with Henoch-Schönlein syndrome (HSS). The renal biopsies revealed AA-type amyloidosis in all three cases. Case 2 displayed focal and segmental proliferative glomerulonephritis in the same renal biopsy. No evidence of well-known diseases and/or conditions for the development of AA-type amyloidosis except for familial Mediterranean fever (FMF) existed in these particular cases. On the other hand, the frequency of the association between FMF and HSS has been reported extensively in the literature; thus, common etiological factors can be considered. The mechanism involved in amyloid deposition in these cases may be related to HSS-associated chronic antigenemia and/or FMF through a mechanism that is, to date, unknown. Further studies are needed to clarify this causal relationship.

Adolescent↗
PubMed1994

[Ring-cell immunocytoma with polyneuropathy associated with dysgammaglobulinemia and amyloidosis].

Dysgammaglobulinemia, amyloidosis and generalization of tumour occurred step by step in sensomotoric polyneuropathy complicating a nonspecified malignant lymphoma. The tumor was specified later as a IgM lambda immunocytoma with substantial participation of signet ring cells. Their vacuoles were sometimes multivesicular and did not contain immunoglobulins. Final phase of the disease was characterized by nodular AL amyloidosis expressed especially in the lung, retroperitoneum and nervous system. Amyloidosis was connected with the vessel walls and their surroundings. Possible autoimmune pathogenesis of polyneuropathy as well as of immunocyte vacuolization were discussed.

Adult↗
PubMed1976

Amyloid fibril protein AA in Papua New Guinean amyloidosis.

In this study of protein composition of amyloid fibrils isolated from eight patients representative of the spectrum of amyloidosis found in Papua New Guinea has been investigated. All fibril preparations, including three from patients with amyloidosis secondary to lepromatous leprosy and one from an unusual juvenile case of primary amyloidosis, contained the non-immunogobulin amyloid protein, protein AA. However, only 44% of thirty-six amyloid patients had detectable levels of the protein AA-related serum component, protein SAA. Alkali-degraded material from each of the fibril preparations failed to react in double immunodiffusion test with antiserum to the amyloid-related light chain VgammaV, but evidence was found for this immunoglobulin light chain-specificity in the serum of one patient.

Adult↗
PubMed1994

Familial nephropathic systemic amyloidosis caused by apolipoprotein AI variant Arg26.

A point mutation in the apolipoprotein AI (apoAI) gene causing autosomal dominant non-neuropathic systemic amyloidosis is described in a previously unreported Canadian family of British origin with five affected individuals in three generations. Amyloid deposits in the renal biopsy from the proband, a 31-year-old female presenting with hypertension and renal failure, stained immunospecifically with antiserum to apoAI. The plasma of all family members with amyloidosis contained both wild-type apoAI and a variant bearing one additional positive charge. Sequencing of the apoAI gene demonstrated that the proband was a heterozygote for a single base substitution in exon 3, changing codon 26 from GGC(Gly) to CGC(Arg). Concordance of the mutant allele with the presence of variant plasma apoAI and clinical features of amyloidosis was demonstrated. This is the third family in which this amyloidotic mutation has been described, but the distribution of amyloid deposits and their clinical effects are clearly determined by other genetic and/or environmental factors.

Adult↗
PubMed1993

[Postmortem diagnosis of cardiac amyloidosis in the aged. Anatomoclinical correlation].

The absence of specific clinical signs makes the diagnosis of cardiac amyloidosis difficult. Moreover, it is established that this condition, the prevalence of which increases with age, aggravates the prognosis of cardiac failure. The present study was undertaken to identify the clinical or paraclinical signs enabling more accurate diagnosis of this disease. Analysis of 2589 autopsy reports from the University Institutes of Geriatrics of Geneva between January 1972 and January 1990 recensed 58 cases of microscopic cardiac amyloidosis, but this diagnosis was not made in any of these patients before death. Of the potential indicators, the good specificity but poor sensitivity of atrial fibrillation and low voltage electrocardiogram was confirmed. On the other hand, the author's research found the association of radiological cardiomegaly and a raised erythrocyte sedimentation rate in nearly 70% of cases of cardiac amyloidosis with a false positive rate of only 10% in a control group.

Aged↗
PubMed1993

[Cardiac amyloidosis: an analysis of five cases].

Five patients with the diagnosis of cardiac amyloidosis established by autopsy and biopsy of noncardiac tissue were reported. All of the patients had cardiac dysfunction. Echocardiogram showed symmetrical thickness of left ventricle, increase of heart weight and reduction of left ventricular ejection fraction. Only two cases had "granular sparkling" on the ventricular wall. The clinical findings of cardiac amyloidosis are arrhythmias, conduction disturbances and intractable heart failure. Echocardiographic examination may reveal cardiac involvement and severity of pathological involvement and predict prognosis. The "granular sparkling" is not a specific feature for the diagnosis of cardiac amyloidosis. Biopsy of either noncardiac or cardiac tissue is required for correct diagnosis.

Amyloidosis↗
PubMed1993

Ultrasonographic detection of thickened joint capsules and tendons as marker of dialysis-related amyloidosis: a cross-sectional and longitudinal study.

Dialysis-related amyloidosis is characterized by a beta 2-microglobulin (beta 2M) infiltration of joint synovia, tendons and capsules. We report a cross-sectional ultrasonographic evaluation of supraspinatus tendon and femoral neck capsule thickness in 49 patients on long-term haemodialysis. Ultrasonographic evaluation was repeated 21 +/- 4 (SD) months later in 16 patients. Normal values for the supraspinatus tendon and femoral neck capsule were defined in a group of control subjects without history or signs of joint disease. Among the 49 patients, aged 21-86 (median 59) years, dialysed for 1-228 (median 97) months, 33 had at least one abnormal joint. The prevalence of patients with at least one and at least two abnormal joints, the number of abnormal joints per patient, and the thickness of the supraspinatus tendon and femoral neck capsule increased significantly with dialysis duration (P < 0.001 for all parameters considered). By multiple linear regression analysis, mean thickness of the supraspinatus tendon was positively related to both dialysis duration (P < 0.0001) and age (P = 0.036) independently. All (n = 11) patients with radiological and/or histological evidence of dialysis-related amyloidosis at the time of ultrasonography had thickened supraspinatus tendon and/or femoral neck capsule; which were also thickened in an additional 22 patients without radiological evidence of dialysis-related amyloidosis. Three died within 5-10 months of the ultrasonographic investigation: post-mortem examination of the periarticular tissue confirmed that the detected thickening was due in all three to beta 2M amyloid infiltration.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1993

Husbandry factors and the prevalence of age-related amyloidosis in mice.

A retrospective study of the prevalence of amyloidosis in mice from several facilities was done. Amyloid deposition is an age-related lesion. The influence of common laboratory factors on the occurrence of this lesion was analyzed. This study documented genotypic difference in susceptibility to amyloidosis and showed that caging and pathogen status both impact on the number of cases of amyloidosis seen in a population. The lowest percentage of affected mice was seen when the animals were individually caged in a specific pathogen-free facility where conditions of stress were minimized.

Age Factors↗