MyMedR

Fulltext Abdominal subcutaneous fat aspiration--an alternative method to diagnose amyloidosis

Ong SG, Rajasingam R

Med J Malaysia, 2007 Mar;62(1):68-9.

A middle aged lady presented with clinical manifestations of primary amyloidosis which included amyloid cardiomyopathy. There was failure to confirm the diagnosis of amyloidosis with biopsies from rectal and tongue tissues. Fat tissue obtained from abdominal subcutaneous fat aspiration eventually demonstrated the presence of amyloid.

Matched MeSH terms: Amyloidosis/diagnosis*; Amyloidosis/physiopathology

Lichen amyloidosis

Dutt AK

Med J Malaya, 1969 Dec;24(2):164-5.

PMID: 4244146

Matched MeSH terms: Amyloidosis*

The epidemiology of cutaneous amyloidosis in Jakarta (Indonesia)

Djuanda A, Wiryadi BE, Sularsito SA, Hidayat D

Ann Acad Med Singap, 1988 Oct;17(4):536-40.

PMID: 3265605

An analysis of cutaneous amyloidosis cases during a five year period, from January 1, 1983 to December 31, 1987, showed that of 78 cases suffering from lichen amyloidosis, 9 patients (11.5%) were male and 71 patients (80.5%) female. Macular skin amyloidosis was observed. Sixty patients (76.9%) were found in the age range of 20-50 years. Forty-nine patients (62.8%) suffered from the disease for 2 years or less. Skin changes were mainly located on the shin areas, the posterior part of the lower thighs and posterior part of the forearms. Treatment with strong topical corticosteroids and keratolytic agents (salicylic acid ointment in higher than 3%) proved to be unsatisfactory. The literature mentions higher incidence of lichen amyloidosis in Chinese, Malaysians and Indonesians than in Caucasians.

Matched MeSH terms: Amyloidosis/epidemiology*; Amyloidosis/pathology

Fulltext The pattern of amyloid deposition in the lung

Looi LM

Malays J Pathol, 1999 Jun;21(1):29-35.

PMID: 10879276

A review of routine histopathological samples and autopsies examined at the Department of Pathology, University of Malaya revealed 15 cases of amyloidosis of the lung. Two were localized depositions limited to the lung while in the remainder, lung involvement was part of the picture of systemic amyloidosis. Both cases of localized amyloidosis presented with symptomatic lung/bronchial masses and a clinical diagnosis of tumour. Histology revealed "amyloidomas" associated with heavy plasma cell and lymphocytic infiltration and the presence of multinucleated giant cells. In both cases, the amyloid deposits were immunopositive for lambda light chains and negative for kappa chains and AA protein. One was a known systemic lupus erythematosus patient with polyclonal hypergammaglobulinaemia. The other patient was found to have plasma cell dyscrasia with monoclonal IgG lambda gammopathy. Both patients did not develop systemic amyloidosis. In contrast, lung involvement in systemic AA amyloidosis was not obvious clinically or macroscopically but was histologically evident in 75% of cases subjected to autopsy. Amyloid was detected mainly in the walls of arterioles and small vessels, and along the alveolar septa. It was less frequently detected in the pleura, along the basement membrane of the bronchial epithelium and around bronchial glands. In one case of systemic AL amyloidosis associated with multiple myeloma, an "amyloidoma" occurred in the subpleural region reminiscent of localized amyloidosis. These cases pose questions on (1) whether localized "tumour-like" amyloidosis is a forme fruste of systemic AL amyloidosis and (2) the differing pattern of tissue deposition of different chemical types of amyloid fibrils, with the suggestion that light chain amyloid has a greater tendency to nodular deposition than AA amyloid.

Matched MeSH terms: Amyloidosis/metabolism*

Fulltext Localised nodular pulmonary amyloidosis in a patient with sicca syndrome

Srinivas P, Liam CK, Jayaram G

Med J Malaysia, 2000 Sep;55(3):385-7.

PMID: 11200724

A 52 year old Chinese woman with a 25 year history of sicca syndrome (primary Sjogrens syndrome) was investigated for 3 episodes of haemoptysis. Clinical examination was unremarkable except for the presence of dry eyes and xerostomia. Computed tomography of the chest revealed a lobulated mass in the posterior basal segment of the left lower lobe. Histopathological examination of this resected nodule confirmed the diagnosis of nodular amyloidosis. The normal radiolabelled serum amyloid P component scintigraphy and the absence of monoclonal plasma cell dyscrasia in the bone marrow strongly support the diagnosis of localised nodular pulmonary AL amyloidosis in this patient. Nodular pulmonary amyloidosis can be associated with sicca syndrome and often simulates bronchogenic carcinoma, bronchiectasis or pulmonary tuberculosis.

Matched MeSH terms: Amyloidosis/complications*; Amyloidosis/pathology; Amyloidosis/radiography

Morphologic differences in the pattern of liver infiltration between systemic AL and AA amyloidosis

Looi LM, Sumithran E

Hum Pathol, 1988 Jun;19(6):732-5.

PMID: 2454214

Biopsy and necropsy tissue from 31 unselected patients with systemic amyloidosis, in which there was histologic evidence of liver involvement, were reviewed with reference to the location and pattern of amyloid deposition in the liver. Amyloidosis was classified into AA and AL types on the basis of immunohistochemistry and permanganate reaction of the amyloid deposits. Nineteen were categorized as AA (secondary) and 12 as AL (primary) amyloidosis. Deposition of AA amyloid was limited to the walls of vessels in the portal tract, constituting a "vascular" pattern. In AL amyloidosis, the deposits exhibited a "sinusoidal" pattern in that they were seen along hepatic sinusoids as well as in vessel walls. This difference was statistically significant (P less than .001). The histologic pattern of liver infiltration offers a valuable clue in the classification of systemic amyloidosis and provides information that may be useful in the selection of patients for therapy.

Matched MeSH terms: Amyloidosis/classification; Amyloidosis/metabolism; Amyloidosis/pathology*

Echocardiographic and electrocardiographic presentations of patients with endomyocardial biopsy-proven cardiac amyloidosis

Roslan A, Kamsani SH, Nay TW, Tan KL, Hakim N, Tan AM, et al.

Med J Malaysia, 2018 12;73(6):388-392.

PMID: 30647209

OBJECTIVE: Cardiac amyloidosis is under diagnosed and its prevalence is unknown. This is a retrospective, nonrandomised, single centre study of patients with endomyocardial biopsy-proven cardiac amyloidosis focusing on their echocardiographic and electrocardiogram (ECG) presentations. This is the first case series in Malaysia on this subject.

METHODS: We identified all of our endomyocardial biopsyproven cardiac amyloidosis patients from January 2010 to January 2018 and reviewed their medical records. All patients echocardiographic and ECG findings reviewed and analysed comparing to basic mean population value.

RESULTS: In total there are 13 biopsy-proven cardiac amyloidosis patients. All of the biopsies shows light chain (AL) amyloid. Majority of the patients (8, 61.5%) is male, and most of our patients (8, 61.5%) is Chinese. All seven patients on whom we performed deformation imaging have apical sparing pattern on longitudinal strain echocardiogram. Mean ejection fraction is 49.3%, (SD=7.9). All patients have concentric left ventricular hypertrophy and right ventricular hypertrophy. Diastolic dysfunction was present in all of our patients with nine out of 13 patients (69.2%) having restrictive filling patterns (E/A ≥2.0 E/e' ≥15). On electrocardiogram, 12 (92%) patients have prolonged PR interval (median 200ms, IQR 76.50ms) and 9 (69.2%) patients have pseudoinfarct pattern.

CONCLUSION: Echocardiography plays an important role in diagnosing cardiac amyloidosis. The findings of concentric left ventricular hypertrophy with preserved ejection fraction without increased in loading condition should alert the clinician towards its possibility. This is further supported by right ventricular hypertrophy and particularly longitudinal strain imaging showing apical sparing pattern.

Matched MeSH terms: Amyloidosis/diagnosis; Amyloidosis/pathology; Amyloidosis/physiopathology*

Fulltext Renal-limited AL amyloidosis - a diagnostic and management dilemma

Fuah KW, Lim CTS

BMC Nephrol, 2018 11 06;19(1):307.

PMID: 30400895 DOI: 10.1186/s12882-018-1118-8

BACKGROUND: Amyloidosis is a disorder caused by extracellular tissue deposition of insoluble fibrils which may result in a wide spectrum of symptoms depending upon their types, sites and amount of deposition. Amyloidosis can be divided into either systemic or localized disease.

CASE PRESENTATION: We present a case of a middle-aged gentleman who presented with persistent nephrotic syndrome with worsening renal function. Repeated renal biopsies showed the presence of renal-limited AL amyloidosis. Systemic amyloidosis workup was unremarkable apart from a slightly raised band of IgG lambda level with no associated immunoparesis. The nephrotic syndrome and renal histology did not improve over a 3-year period despite being given two courses of chemotherapies.

CONCLUSION: We hope that early recognition of this unusual localised presentation of renal- limited AL Amyloidosis and its poor response to conventional treatment can alert the nephrologist to the potential existence of this rare condition.

Matched MeSH terms: Amyloidosis/blood*; Amyloidosis/diagnosis*; Amyloidosis/therapy

Transthyretin Glu18. A new variant associated with familial amyloid polyneuropathy

Tan SY, Hawkins PN, Pepys MB

JUMMEC, 1998;3:54-55.

Familial amyloid polyneuropathy is most commonly associated with variant plasma transthyretin (TTR) although it has been described in association with variant apolipoprotein A1 and gelsolin. More than 40 TTR variants, all consisting of single amino acid substitutions diskibuted widely along the length of the 127 residue TTR subunit have now been described. We report here a novel TTR variant, Glu18, in a Colombian woman with TTR amyloidosis.

Matched MeSH terms: Amyloidosis

An immunohistochemical and morphological study of amyloidosis complicating leprosy in Malaysian patients

Looi LM, Jayalakshim P, Lim KJ, Rajagopalan K

Ann Acad Med Singap, 1988 Oct;17(4):573-8.

PMID: 3223746

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.

Matched MeSH terms: Amyloidosis/etiology*; Amyloidosis/metabolism; Amyloidosis/epidemiology; Amyloidosis/pathology

Primary cardiac amyloidosis

Jeyamalar R, Pathmanathan R, Yap SF, Kannan P

Ann Acad Med Singap, 1991 Nov;20(6):795-7.

PMID: 1803972

Cardiac amyloidosis is an uncommon and often unrecognised cause of cardiac failure. It is an infiltrative disease that may mimic either a restrictive or hypertrophic cardiomyopathy, constrictive pericarditis, coronary artery disease or valvular heart disease. The diagnosis should be suspected in a patient with cardiac failure who has low voltage complexes on the electrocardiogram, in association with increased myocardial mass and echogenicity on the echocardiogram. The definitive diagnosis, however, can only be made by endomyocardial biopsy or biopsy of any involved organ in systemic amyloidosis. Prognosis is poor and treatment ineffective.

Matched MeSH terms: Amyloidosis/diagnosis*

Familial lichen amyloidosis. Report of 19 cases in 4 generations of a Chinese family in Malaysia

Rajagopalan K, Tay CH

Br J Dermatol, 1972 Aug;87(2):123-9.

PMID: 5057380 DOI: 10.1111/j.1365-2133.1972.tb16186.x

Matched MeSH terms: Amyloidosis/genetics*

The pattern of amyloidosis in Malaysia

Looi LM

Malays J Pathol, 1994 Jun;16(1):11-3.

PMID: 16329569

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.

Matched MeSH terms: Amyloidosis/etiology; Amyloidosis/epidemiology; Amyloidosis/pathology*

Intratumour amyloidosis in Malaysians: an immunohistochemical study

Looi LM

Ann Acad Med Singap, 1986 Jan;15(1):52-6.

PMID: 3010797

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.

Matched MeSH terms: Amyloidosis/complications; Amyloidosis/metabolism; Amyloidosis/pathology*

Microscopical atrial amyloidosis in chronic heart disease

Looi LM

Histopathology, 1981 Nov;5(6):615-22.

PMID: 7319480

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.

Matched MeSH terms: Amyloidosis/etiology*; Amyloidosis/epidemiology; Amyloidosis/pathology

The pattern of amyloidosis in a Malaysian patient population

Looi LM

Histopathology, 1991 Feb;18(2):133-41.

PMID: 1901294

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.

Matched MeSH terms: Amyloidosis/metabolism*; Amyloidosis/epidemiology; Amyloidosis/pathology*

Fulltext Aerodigestive amyloidosis presenting as acute asthma

Sreetharan SS, Prepageran N, Razak A, Valuyeetham KA

Med J Malaysia, 2003 Jun;58(2):290-3.

PMID: 14569753

Aerodigestive amyloidosis is a rare disorder characterized by fibrillar protein deposition in the aerodigestive tree. We present a case of a 19-year-old Chinese gentleman whose diagnosis was initially missed as he presented with features suggestive of severe bronchial asthma and was intubated and ventilated. He subsequently presented 2 years later with severe stridor and required emergency tracheostomy. Current literature is reviewed for the histopathology, common clinical features, radiological findings and treatment options for aerodigestive amyloidosis.

Matched MeSH terms: Amyloidosis/complications; Amyloidosis/diagnosis*; Amyloidosis/therapy

Amyloidosis in Malaysian aborigines (Orang Asli)

Looi LM, Prathap K

Pathology, 1979 Oct;11(4):575-82.

PMID: 93739

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.

Matched MeSH terms: Amyloidosis/genetics; Amyloidosis/epidemiology*; Amyloidosis/pathology*

Fulltext Haemodialysis associated amyloidosis of the tongue: a case report

Sathasivam, Hans Prakash, Lau, Shin Hin

Archives of Orofacial Sciences, 2013;8(2):65-68.

MyJurnal

Haemodialysis associated amyloidosis (HAA) is a complication of long-term haemodialysis caused by deposition of β2-microglobulin in tissues that most often presents clinically at osteoarticular sites. However, in very rarecircumstances, patients do present initially with oral manifestations of HAA. In a normally functioning kidney, β2-microglobulin is cleared by glomerular filtration and is catabolized in the proximal tubules. This article describes a patient with oral manifestation of haemodialysis associated amyloidosis with an unusual presenting complaint of lingual dysaesthesia.

Matched MeSH terms: Amyloidosis

Fulltext Cerebrospinal fluid rhinorrhoea secondary to amyloidosis of the sphenoid sinus

Ali EA, Philip R, Prepageran N, Peh SC

Med J Malaysia, 2008 Oct;63(4):341-2.

PMID: 19385501 MyJurnal

Amyloidosis of the skull base is a rare entity. A patient with localized amyloidosis of the sphenoid sinus presented at our institution with cerebrospinal fluid rhinorrhoea. Endoscopic excision of the lesion and multilayered obliteration of the sphenoid sinus resolved the symptoms.

Matched MeSH terms: Amyloidosis/complications*; Amyloidosis/surgery

Displaying publications 1 - 20 of 34 in total