exudates and Amyloidosis

Congo red screening of routine biopsies at the University Hospital Kuala Lumpur revealed the following categories of amyloidosis: systemic AL (5.9%); systemic AA (3.2%); isolated atrial (14%); primary localized cutaneous (7.5%); other primary localized deposits (3.2%); localized intratumour (58%); and dystrophic (8.6%). Unlike in the West, AA amyloidosis in this population was usually secondary to leprosy or tuberculosis. Liver involvement in AL amyloidosis was shown to exhibit a sinusoidal pattern and differed from the vascular pattern of AA amyloidosis. Within the category of AA amyloidosis, there were two patterns of renal involvement--glomerular and vascular, with the glomerular pattern carrying a more ominous clinical picture. Notable among the localized amyloidoses were isolated atrial amyloidosis complicating chronic rheumatic heart disease, intratumour amyloidosis within nasopharyngeal carcinomas and dystrophic amyloidosis which occurred in fibrotic tissues.

Topics: Adolescent; Adult; Age Distribution; Aged; Aged, 80 and over; Amyloid; Amyloidosis; Biopsy; Child; Humans; Kidney Diseases; Leprosy; Liver Diseases; Malaysia; Middle Aged; Prevalence; Tuberculosis

Congo red screening of 211 consecutive cardiac biopsy specimens obtained during cardiac surgery from 167 patients revealed 26 (16%) instances of isolated atrial amyloidosis (IAA). The ages of IAA-positive patients ranged from 25 to 52 years (mean age, 39 years). Twenty-three (88%) IAA-positive biopsy specimens were from patients with chronic rheumatic heart disease (CRHD) while three (12%) were from patients with an atrial septal defect (ASD). The prevalence of IAA in the CRHD patients was 23%, appreciably higher than that in the ASD patients (15%) and in other patients with atrial biopsies. The prevalence of IAA in both CRHD and ASD patients was significantly higher (P < .001) than in controls. Controls consisted of 247 healthy adults who were autopsied after traumatic deaths, with an age range of 18 to 89 years (mean age, 38 years). Only seven (3%) control subjects were IAA positive; all were over 40 years of age. Isolated atrial amyloidosis deposits were permanganate resistant and immunohistochemically positive for human amyloid P (AP) protein and negative for human amyloid-associated (AA) protein and immunoglobulin light chains. They were observed as fine congophilic and birefringent deposits in intramyocardial vessel walls, along the myocardial sarcolemma, and in the subendocardium. There was associated myocyte hypertrophy but no atrophy. Electron microscopy demonstrated typical nonbranching amyloid fibrils. It is postulated that stretching of the atria in chronic heart disease results in a raised prevalence of IAA. Recent reports that IAA contains atrial natriuretic peptide, a polypeptide hormone product of atrial myocytes, supports this view.

Topics: Adolescent; Adult; Aged; Amyloidosis; Cardiomyopathies; Child; Chronic Disease; Female; Heart Diseases; Heart Valve Diseases; Humans; Malaysia; Male; Microscopy, Electron; Middle Aged; Postoperative Complications; Prevalence; Rheumatic Heart Disease

Congo red screening of 27,052 routine biopsy specimens from 22,827 patients over a 5 1/2-year period in the Department of Pathology, University of Malaya detected 186 cases of amyloidosis. The categories of amyloidosis encountered and their prevalences in relation to each other were: systemic AL (5.9%); systemic AA (3.2%); isolated atrial (14%); primary localized cutaneous (7.5%); other primary localized deposits (3.2%); localized intratumour (58%); and dystrophic (8.6%). A third of patients with systemic AL amyloidosis had coexistent immunocyte abnormality. The commonest underlying pathology for systemic AA amyloidosis was leprosy. Notable among the types of localized amyloidosis revealed by this study were isolated atrial amyloidosis, which appeared to complicate chronic rheumatic heart disease, and intratumour amyloidosis complicating nasopharyngeal carcinoma. Other tumours in which amyloid deposits were observed included basal cell carcinoma, islet cell tumour and medullary carcinoma of the thyroid. Dystrophic amyloidosis was observed in fibrotic tissues, such as damaged cardiac valves and osteoarthritic joints. Heredofamilial amyloidosis, senile systemic amyloidosis and degenerative cerebral amyloidosis were notably absent from this study.

Topics: Adolescent; Adult; Aged; Aged, 80 and over; Amyloid; Amyloidosis; Cardiomyopathies; Child; Humans; Immunoglobulin kappa-Chains; Immunoglobulin lambda-Chains; Immunohistochemistry; Malaysia; Microscopy, Electron; Middle Aged; Neoplasms; Potassium Permanganate; Prevalence; Skin Diseases

A review of consecutive biopsies from 85 Malaysian patients with primary localised cutaneous amyloidosis (PLCA) revealed 63 with papular amyloidosis (PA) and 22 with macular amyloidosis (MA). PLCA appeared to affect the Chinese more frequently than the other major ethnic groups but MA was more common than expected among the Indians. Of patients with PA, one had systemic lupus erythematosus, one scleroderma and in another, connective tissue disease was suspected. MA was not found to be associated with any other disease. Histologically, PA differed from MA by the larger size of amyloid deposits in the papillary dermis. There was no difference in their tinctorial and immunohistochemical characteristics. Deposits were permanganate-resistant and negative for AA protein, immunoglobulin light chains and keratin. A few cases exhibited positively for cytokeratin. Strong immunoreactivity for AP protein was observed. PA and MA appear chemically similar and are likely to be of epidermal origin.

Topics: Adolescent; Adult; Aged; Amyloidosis; Child; China; Female; Humans; Immunohistochemistry; Malaysia; Male; Middle Aged; Serum Amyloid A Protein; Skin Diseases

Congo red screening of tissue blocks from 37 consecutive autopsies on leprosy patients revealed 7 cases of systemic amyloidosis, indicating a prevalence rate of 19%. 5 were males and 2 females. All were ethnic Chinese. Their ages ranged from 52 to 85 years with a mean of 69 years. Six had lepromatous leprosy while the remaining 1 had tuberculoid leprosy. In all 7 cases, the amyloid was AA in type, being permanganate-sensitive and immunoreactive with anti-human AA protein antiserum. Hepatic deposition was limited to blood vessels, a pattern typical of AA (secondary) amyloidosis. With regard to renal involvement, 4 showed a predominantly vascular pattern of infiltration while 3 exhibited the more ominous glomerular pattern. Three died of chronic renal failure and 2 of congestive cardiac failure attributable to renal and cardiac amyloidosis respectively. One patient succumbed to septicaemia and the remaining 1 to acute myocardial infarction. AA amyloidosis remains a serious and significant complication of leprosy among Malaysians.

Topics: Aged; Aged, 80 and over; Amyloidosis; China; Female; Humans; Leprosy, Borderline; Leprosy, Lepromatous; Leprosy, Tuberculoid; Malaysia; Male; Middle Aged; Serum Amyloid A Protein

The findings of autopsies performed on 35 leprosy subjects in the University Hospital, Kuala Lumpur, between January 1981 and December 1985 are presented. This is the first report based on autopsy findings from Malaysia. The patients were elderly subjects with a mean age of 74 years. Sixty-six percent had lepromatous leprosy. None had active skin lesions. The most common cause of death was pyogenic infection, particularly bronchopneumonia. Tuberculosis was noted in 25% of the cases. The other important causes of death included cardiac and renal failure. Renal lesions were evident in 71% of the cases, and the most common pathology was interstitial nephritis. Generalized amyloidosis complicated six (17%) patients.

Topics: Aged; Aged, 80 and over; Amyloid; Amyloidosis; Bronchopneumonia; Female; Heart Diseases; Humans; Kidney; Leprosy; Malaysia; Male; Middle Aged; Myocardium; Neoplasms; Peripheral Nerves; Skin; Tuberculosis, Pulmonary

Congo red screening of tumour material examined at the Department of Pathology, University of Malaya revealed intratumour deposits of amyloid in 12% of nasopharyngeal carcinomas, 66% of basal cell carcinomas, 100% of medullary carcinomas of the thyroid, 56% of islet cell tumours of the pancreas, 1 out of 16 carcinoids and 1 out of 100 thyroid adenomas. All the deposits were permanganate resistant and did not contain AA protein, indicating that what was encountered was not secondary amyloid. The deposits showed variable staining for immunoglobulin light chains and amyloid P component with a standard peroxidase antiperoxidase method. The possibility that intratumour amyloid has a neoplastic origin is discussed.

Topics: Adenoma, Islet Cell; Amyloid; Amyloidosis; Carcinoid Tumor; Carcinoma, Basal Cell; Histocytochemistry; Humans; Immunochemistry; Malaysia; Nasopharyngeal Neoplasms; Neoplasms; Pancreatic Neoplasms; Thyroid Neoplasms

Congo-red screening demonstrated intratumor deposits of amyloid in 35 of 53 unselected cases of basal cell carcinoma. Male subjects had a higher amyloid positivity rate than female subjects. The amyloid deposits were permanganate-resistant and located in the stroma between clumps of tumor cells, as well as abutting the advancing front of the neoplasm. Solar elastosis was often observed in the overlying and adjacent subepidermis. The relationship between amyloid positivity and the different histological subtypes of basal cell carcinoma, tumor ulceration, and density of the lymphoplasmacytic stromal infiltrate were also studied. The possibility that amyloid originates from the tumor cells and is a result of tumor apoptosis (degeneration) is discussed.

Topics: Amyloid; Amyloidosis; Carcinoma, Basal Cell; Congo Red; Female; Humans; Malaysia; Male; Microscopy, Electron; Racial Groups; Sex Factors; Skin Neoplasms; Time Factors

Nineteen out of 121 consecutive cardiac biopsies from 107 patients were found to contain amyloid deposits on routine Congo red screening. Seventeen were left atrial appendages removed during mitral valvotomy for chronic rheumatic mitral valve disease while the remaining two were right atrial appendages excised during surgical repair of atrial septal defects. The distribution of amyloid deposits within the atria and their tinctorial characteristics are described. The high prevalence of atrial amyloidosis observed could not be attributed to generalized or senile amyloidosis. The possibility that this is a distinctive localized form of amyloidosis secondary to chronic heart disease is discussed.

Topics: Adolescent; Adult; Aged; Amyloidosis; Child; Chronic Disease; Female; Heart Diseases; Heart Septal Defects, Atrial; Humans; Malaysia; Male; Middle Aged; Myocardium; Rheumatic Heart Disease

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.

Topics: Adult; Aged; Amyloidosis; Asian People; Black People; Female; Humans; Kidney; Liver; Malaysia; Male; Middle Aged; Myocardium; Racial Groups; Spleen; Staining and Labeling

Topics: Adult; Amyloidosis; Ethnicity; Female; Humans; Malaysia; Male; Middle Aged; Tuberculosis, Pulmonary

Topics: Amyloid; Amyloidosis; Brazil; England; Humans; Malaysia; Portugal; Scandinavian and Nordic Countries