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PubMed1982

Generalised pustular psoriasis, psoriatic arthritis and nephrotic syndrome associated with systemic amyloidosis.

The case report is presented of a psoriatic patient with arthropathy, generalised pustular psoriasis and nephrotic syndrome, in whom systemic amyloidosis developed. The literature reports 13 cases of psoriasis associated with amyloidosis, 3 of whom suffered from pustular psoriasis as does our case. With the addition of our case, 12 of these 14 had concomitant arthropathy. This seems to suggest that arthritis is an important factor in the appearance of amyloidosis. Rectal biopsy and/or renal biopsy may be helpful in establishing the diagnosis of amyloidosis relatively early in patients with psoriatic arthritis.

Amyloidosis↗
PubMed1991

Unilateral extended amyloidosis of the renal pelvis and ureter: a case report.

Localized amyloidosis of the genitourinary tract is a rare phenomenon. The differential diagnosis between amyloidosis and malignancy is difficult. We report the case of an 81-year-old man with extensive unilateral localized amyloidosis of the renal pelvis, ureter and ureteral orifice. Diagnosis of amyloidosis was confirmed only postoperatively.

Aged↗
PubMed1977

Secondary glaucoma accompanied with primary familial amyloidosis.

22 patients were diagnosed as having primary familial amyloidosis by the histological examination of biopsy specimens, clinical course of the disease, and family history, having a focus in the Nagano Prefecture in Japan. All patients belonged to the Andrade type of the neuropathy form in the primary familial amyloidosis. 15 out of 22 patients underwent ophthalmological examination, and secondary glaucoma, due to primary familial amyloidosis, was found in 4 patients. The clinical characteristic features of the secondary glaucoma were described as follows: (1) having the systemic symptoms of primary familial amyloidosis; (2) belonging to relatively older age groups and having a longer duration of the course of the disease; (3) having vitreous opacities; (4) having the wide open angle with pigment deposition in the gonioscopical examination; (5) having white flocculent materials in the pupillary margin, and (6) having flaky substances on the surface of the lens. The ophthalmological symptoms mentioned in 4, 5, and 6 were very similar to those of glaucoma capsulare. The tissue specimens obtained at the time of antiglaucoma surgery were subjected to light and electron microscopy. Amyloid fibrils were found in the iris and trabeculum, and the pathogenesis of secondary glaucoma was speculated to be a deposition of amyloid fibrils in the trabecular meshwork.

Adult↗
PubMedDate not supplied

Urine cytology of localized primary amyloidosis of the ureter: a case report.

BACKGROUND: Morphologic findings of amyloid in urine cytology material have rarely been reported because amyloidosis of the urinary tract is a relatively uncommon disorder. We present a case of primary amyloidosis of the ureter, including catheterized urine cytologicfindings. CASE: A 78-year-old man had pollakiuria and dysuria for 5 years before admission after transurethral resection of the prostate. Clinical examination revealed left hydronephrosis and stricture of the lower part of the left ureter, and a malignant ureteral tumor was suspected clinically. In catheterized urine cytology, many clusters of epithelial cells, inflammatory cells and abundant, amorphous, waxy material were observed. The amorphous material stained light green by the Papanicolaou method and positive with direct fast scarlet (DFS), showing yellow-green birefringence under polarized light. Positivity with DFS staining was not affected by treatment with potassium permanganate. Immunocytochemically the material was AL-type amyloid protein. Atypia were absent from epithelial cells. The patient had no history of diseases that could cause secondary amyloidosis. The present case was considered to be primary amyloidosis localized to the left ureter because no particular morphologic change in the epithelial cells of the urinary tract was observed. CONCLUSION: Amyloid can be present in urine and should not be overlooked or confused with tumor diathesis when a malignant tumor is suspected clinically.

Aged↗
PubMedDate not supplied

Localized amyloidosis in nasopharyngeal carcinoma diagnosed by fine needle aspiration and electron microscopy. A case report.

BACKGROUND: The association of amyloidosis and tumors is well known, but only rarely has it been found in the nasopharynx. Moreover, only a single case of tumor-associated amyloidosis in the nasopharynx has been diagnosed by exfoliative cytology and reported in the English-language literature. We describe a case of localized amyloidosis in metastatic nasopharyngeal carcinoma diagnosed by fine needle aspiration and confirmed by histology and electron microscopy. CASE: Bilateral neck enlargement appeared six months before consultation in a 57-year-old man. Both masses were resected, and during exploration of the nasopharynx, several blind biopsies were taken. Smears from the fine needle aspirate showed malignant epithelial cells, consistent with metastatic carcinoma, and Congo red stain showed the presence of amyloid. Histologic examination of the resected cervical masses and nasopharyngeal biopsies confirmed the diagnosis of nasopharyngeal carcinoma, nonkeratinizing variant with amyloid, and metastases in two lymph nodes. Electron microscopic examination of a lymph node showed extracellular, ribbon-like amyloid deposits as well as masses of amorphous amyloid, apparently intracellular. CONCLUSION: The origin of this form of amyloidosis is unclear in this patient since he had no other known etiologic factors for the condition. Increased awareness of the potential association of these two conditions and the use of fine needle aspiration may reveal a higher frequency than hitherto reported.

Amyloidosis↗
PubMed2003

Longitudinal myocardial function assessed by tissue velocity, strain, and strain rate tissue Doppler echocardiography in patients with AL (primary) cardiac amyloidosis.

BACKGROUND: AL amyloidosis with heart failure is associated with decreased longitudinal myocardial contraction measured by pulsed tissue Doppler imaging. We sought to clarify whether new modalities of myocardial strain Doppler (change in length per unit length) or strain rate (the temporal derivative of strain) were more sensitive than tissue Doppler and could detect early regional myocardial dysfunction before the onset of congestive heart failure (CHF) in patients with AL (primary) amyloidosis. METHODS AND RESULTS: Ninety-seven biopsy-proven patients with AL amyloidosis were divided into 3 groups. Group 1 patients had no cardiac involvement (n=36), group 2 had heart involvement but no CHF (n=32), and group 3 had heart involvement and CHF (n=29). All patients underwent tissue velocity (TV) imaging, strain, and strain rate imaging (SR) at the basal, mid, and apical ventricle in 2 apical views. With the use of TV, differences in systolic function were only apparent between group 3 (basal mean value, 3.0+/-1.1 cm/s) and groups 1 and 2 (5.0+/-1.3 and 4.6+/-1.2 cm/s, respectively). In contrast, basal peak systolic SR (l/s) showed significant differences among all 3 groups (-2.0+/-0.4, -1.55+/-0.6, and -0.76+/-0.3 for groups 1 to 3, respectively. P<0.01). Basal strain also demonstrated statistically significant differences among the groups (-19+/-4%, -15+/-4.5%, and -8.0+/-5%; P<0.01). CONCLUSIONS: Cardiac amyloidosis is characterized by an early impairment in systolic function at a time when fractional shortening remains normal. This abnormality precedes the onset of CHF and can be detected by strain and SR but is not apparent by TV imaging.

Amyloidosis↗
PubMed1981

M-mode and two-dimensional echocardiographic features in cardiac amyloidosis.

Twenty-eight patients with cardiac amyloidosis were studied by echocardiography -- 26 by M-mode and 13 by two-dimensional (2D) studies. All had heart failure and biopsy-proved amyloidosis, M-mode features included (1) normal left ventricular (LV) dimension in all; (2) thickened ventricular septum (88%), LV posterior wall (77%), and right ventricular (RV) anterior wall (79%); (3) decreased thickening of ventricular septum (96%) and of LV posterior wall (65%) and reduced LV global function (62%); (4) left atrial enlargement (50%); and (5) pericardial effusion (58%). Two-dimensional echocardiography provided additional features: (1) thickened papillary muscles (five of 13); (2) thickened valves (four of 13); (3) better appreciation of thickened RV wall; and (4) a characteristic "granular sparkling" appearance of thickened cardiac walls -- presumably secondary to the amyloid deposit -- which was noted in 12 of 13 patients. Thus, M-mode echocardiography is helpful in the recognition of cardiac amyloidosis. However, the better appreciation with 2D echocardiography of thickened cardiac walls with a "granular sparkling" appearance in patients with unexplained cardiac failure is virtually diagnostic of cardiac amyloidosis.

Adult↗
PubMed1991

Prognostic significance of Doppler measures of diastolic function in cardiac amyloidosis. A Doppler echocardiography study.

BACKGROUND: We have previously characterized the left ventricular diastolic filling abnormalities in cardiac amyloidosis by Doppler methods. The various filling patterns were shown to be related to the degree of cardiac amyloid infiltration. The purpose of this study was to determine the value of Doppler diastolic filling variables for assessing prognosis in cardiac amyloidosis. METHODS AND RESULTS: We performed pulsed-wave Doppler studies of the left ventricular inflow and obtained clinical follow-up data in 63 consecutive patients with biopsy-proven systemic amyloidosis. All patients had typical echocardiographic features of cardiac involvement. The patients were subdivided into two groups according to deceleration time: Group 1 (33 patients) had a deceleration time of 150 msec or less, indicative of restrictive physiology, and group 2 (30 patients) had a deceleration time of more than 150 msec. Of the 63 patients, 32 (51%) died during a mean follow-up period of 18 +/- 12 months. Of these deaths, 25 (78%) were cardiac deaths, and 19 of the 25 patients (76%) were from group 1. The 1-year probability of survival in group 1 was significantly less than that in group 2 (49% versus 92%, p less than 0.001). Bivariate analysis revealed that the combination of the Doppler variables of shortened deceleration time and increased early diastolic filling velocity to atrial filling velocity ratio were stronger predictors of cardiac death than were the two-dimensional echocardiographic variables of mean left ventricular wall thickness and fractional shortening. CONCLUSIONS: Doppler-derived left ventricular diastolic filling variables are important predictors of survival in cardiac amyloidosis.

Amyloidosis↗
PubMedDate not supplied

Immunohistochemical characterization of the amyloid deposits and quantitation of pertinent cerebrospinal fluid proteins in hereditary cerebral hemorrhage with amyloidosis.

Cystatin C, a protein inhibitor of lysosomal cysteine proteinases, was demonstrated by immunohistochemical techniques to be present in the birefringent amyloid deposits of the small arteries in the cerebrum, cerebellum, and leptomeninges of 10 Icelandic individuals with hereditary cerebral hemorrhage with amyloidosis. Specimens from other organs were investigated in one of the patients, and amyloid angiopathy characterized by an immunoreactivity of cystatin C was found in a submandibular lymph node. No immunoreactivity of amyloid fibril protein AA, kappa or lambda immunoglobulin light chain, or prealbumin was observed. Significantly low cerebrospinal fluid concentrations of cystatin C were found in all 9 investigated individuals with hereditary cerebral hemorrhage with amyloidosis. The concentrations of beta 2-microglobulin, albumin, and IgG in the cerebrospinal fluid were within normal limits. Isoelectric focusing showed that cystatin C from the cerebrospinal fluid of 9 patients with hereditary cerebral hemorrhage with amyloidosis had an isoelectric point identical to that of normal individuals. This investigation demonstrates that hereditary cerebral hemorrhage with amyloidosis may be diagnosed by two laboratory methods: immunohistochemical investigation of cystatin C in brain tissue specimens and quantitation of cystatin C in cerebrospinal fluid.

Adult↗
PubMed1990

Metabolic studies of radioiodinated serum amyloid P component in normal subjects and patients with systemic amyloidosis.

125I-Serum amyloid P component (SAP), injected intravenously into 10 normal subjects, remained predominantly intravascular with mean (SD) T1/2 (half time) in plasma of 24.5 (5.9) h. The fractional catabolic rate of 68 (19)% of the plasma pool per day was more rapid than other reported human plasma proteins. All radioactivity was excreted in the urine by 14 d. In 16 patients with monoclonal gammopathy or chronic inflammatory diseases, but without amyloidosis, 125I-SAP metabolism was normal. However, among 45 patients with biopsy-proven systemic amyloidosis (25, amyloid A type; 20, amyloid L type), 125I-SAP was cleared from the plasma more rapidly, accumulated in the amyloid deposits, and persisted there. The T1/2 in amyloid, measured directly with 131I-SAP, was 24 d. Repeat studies after 6-18 mo were notably consistent in normals but changed significantly in amyloid patients, generally correlating with clinical signs of disease progression. Measurements of 125I-SAP turnover may thus be of value for diagnosis and monitoring of amyloidosis. Analysis of SAP metabolism in amyloidosis suggests that plasma SAP is in dynamic equilibrium with a very large amyloid pool, and in two autopsies the total mass of SAP in the amyloid deposits was 2,100 and 21,000 mg, respectively.

Adult↗
PubMed2001

Cardiac amyloidosis presenting as microvascular angina--a case report.

Cardiac amyloidosis usually presents with heart failure, but rarely leads to coronary insufficiency. The authors report a case of a 69-year-old Japanese woman with cardiac amyloidosis presenting as microvascular angina. She had exertional angina with positive exercise test and normal coronary angiograms. However, heart failure developed, and she died 3 years after symptom onset. On autopsy, coronary arteries were patent. In contrast to that of the epicardial coronary arteries, histologic examination of the heart revealed severe obstructive alterations of the intramural coronary arteries with amyloid. Furthermore, amyloid was present mainly in the endocardium and the intramural coronary arteries, but there was little present in the myocardium. This case was a rare AL amyloidosis. There have been only 4 reported cases of cardiac amyloidosis that presented with exertional angina, a positive exercise test, and normal coronary angiograms and histologic examination.

Aged↗
PubMed1994

Amyloidosis of the larynx in a child.

Localized laryngeal amyloidosis is a rare and benign process. Only three cases have been reported in children. We present the case of a 9-year-old girl with localized laryngeal amyloidosis that was excised via laryngofissure. The patient presented with a 3- to 4-week history of progressive hoarseness. Direct laryngoscopy revealed a smooth, firm, submucosal mass extending from the anterior aspect of the left false vocal cord to the vocal process. Biopsy of the mass revealed findings consistent with amyloidosis. The mass was approached via laryngofissure and excised with the thyroid cartilage and left true vocal cord left intact. An extensive workup for systemic amyloidosis was negative, including bone marrow biopsy. The postoperative recovery was uneventful, with good vocal quality and a sluggish but mobile left true vocal cord. This case is significant because of the young age of the child, the extent of disease, and the use of laryngofissure for excision.

Amyloidosis↗
PubMedDate not supplied

Uncommon aetiology in renal amyloidosis.

Nine paraplegic patients due to spinal cord injury of various aetiology, developing renal amyloidosis are presented and the old literature is reviewed. All the patients were male and their mean age was 24.8 +/- 1.5 years. The mean interval between the start of primary disease and the diagnosis of amyloidosis was 2.4 +/- 0.3 years. Five patients (55%) died and four patients (45%) are alive. This study is of interest because of (1) the rarity of renal amyloidosis in paraplegic patients, (2) the short duration between the onset of the primary disease and the amyloidosis, (3) the young age of the patients, (4) and the striking clinical improvement after treatment of the primary disease.

Adolescent↗
PubMedDate not supplied

Pulmonary amyloidosis in familial Mediterranean fever.

Familial Mediterranean Fever (FMF) is a hereditary periodic fever syndrome expressed by acute episodes of fever and painful manifestations. The gravest consequence of FMF is kidney involvement by secondary amyloidosis of AA type, which gradually leads to nephrotic syndrome and uremia. Nephropathic amyloidosis of the AA type, which complicates FMF in most untreated patients, may progress to effect other organs, including the lungs. This kind of organ involvement rarely produces noticeable symptoms and is associated with symptomatic involvement of other organs while remaining subclinical in itself. In this report, one case who had nephropathic and pulmonary amyloidosis of the secondary amyloidosis of AA type, wich complicates the FMF was presented and the pulmonary manifestations of FMF were reviewed.

Adult↗
PubMed2005

Sequential heart and autologous stem cell transplantation for systemic AL amyloidosis.

Extensive cardiac amyloid deposition in systemic AL amyloidosis is associated with a grave prognosis. Heart transplantation is rarely performed because of the systemic and progressive nature of the disease. Patients with severe cardiac amyloid infiltration are ineligible for the preferred treatment of melphalan chemotherapy with stem cell transplantation (SCT) rescue because of the high risk for treatment-related mortality. Heart transplantation followed by SCT was performed in 5 patients with AL amyloidosis and predominant cardiomyopathy. Patients were followed up for a median of 95 months (range, 37-118 months) from diagnosis. At censor, 3 of 5 patients were well without evidence of intracardiac or extracardiac amyloid accumulation, and median overall survival by Kaplan-Meier estimate was not reached. Two patients died of progressive amyloidosis 33 and 90 months after heart transplantation after relapse of their underlying plasma cell dyscrasia. Heart transplantation followed by SCT is feasible in selected patients with cardiac AL amyloidosis and may confer substantial survival benefit.

Adult↗
PubMed1993

Primary systemic amyloidosis: a rare complication of immunoglobulin M monoclonal gammopathies and Waldenström's macroglobulinemia.

PURPOSE: To determine the natural history of amyloidosis associated with Waldenström's macroglobulinemia and immunoglobulin M (IgM) monoclonal gammopathy. PATIENTS AND METHODS: From January 1968 to September 1990, 50 patients with a serum IgM monoclonal protein and biopsy-proven amyloidosis were evaluated at the Mayo Clinic. There were 32 men and 18 women (age range, 43 to 93 years). RESULTS: Percentages of patients presenting with cardiac, renal, hepatic, and pulmonary amyloid were 44%, 32%, 14%, and 10%, respectively. Forty-two percent of the patients had an M protein value greater than 1.5 g/dL, and 12% had an M component greater than 3 g/dL. Subcutaneous fat, rectum, and bone marrow showed amyloid in 84%, 72%, and 50%, respectively, providing a simple technique for diagnosing amyloidosis. The bone marrow biopsy was consistent with Waldenström's macroglobulinemia in 10, a plasma-cell proliferative disorder in 10, and lymphoma or a lymphoproliferative disorder in 11; results were normal, nondiagnostic, or hypercellular in 17. Forty-three of 50 patients died. The median survival of the entire group was 24.6 months. Fifty-three percent of deaths were due to cardiac amyloid, 12% to respiratory failure, 7% to macroglobulinemia, 7% to liver failure, and 7% to kidney failure. CONCLUSION: The presence of amyloid cardiomyopathy and an increased creatinine concentration at diagnosis had an adverse impact on survival. Of the 22 patients who presented with cardiomyopathy, the median survival was 11.1 months, with only two surviving longer than 5 years. The median survival of the 28 patients without cardiomyopathy at diagnosis was 27 months, with eight 5-year survivors (P = .013). All eight amyloid deposits studied stained for Ig light chain, indicating that this amyloidosis is of the primary (AL) type.

Adult↗
PubMed1977

Paneth cells in experimental amyloidosis.

The effect of experimental amyloidosis on the morphology of the Paneth cells of the mouse was investigated by using light and fluorescence microscopy. Amyloidosis was induced by daily subcutaneous casein injections for 4 weeks. It was found that the number of secretory granules of the Paneth cells increased in the jejunum, but remained normal in the duodenum. Small morphological alterations took place in the Paneth cells during the induction of amyloidosis, and occasionally the Paneth cells were totally lost. These changes suggest that the function of the Paneth cells of the jejunum may be partly inhibited during experimental amyloidosis.

Amyloidosis↗
PubMed2004

Management of laryngeal amyloidosis.

Amyloidosis of the upper aerodigestive tract is rare. However, the larynx is the commonest site within the upper airways to be affected. Three cases of uncomplicated primary amyloidosis of the larynx and one case of laryngeal amyloidosis with more generalized dissemination throughout the head and neck are presented. In this article the different presentations of laryngeal amyloidosis and principles of management of this condition at that site are highlighted.

Adult↗