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PubMed1983

[Light and electron microscopy of primary localized cutaneous amyloidosis].

The two polar types of primary cutaneous amyloidosis are characterized by different alterations of the epidermis, i.e., epidermal hyperplasia and hyperkeratosis in lichen amyloidosus, hyperpigmentation without any essential epidermal changes in macular pigmented amyloidosis. Biopsies from six patients suffering from lichen amyloidosus and macular pigmented amyloidosis were examined light- and electron-microscopically to provide any information that could account for the different epidermal behavior in these two polar forms of primary cutaneous amyloidosis. No ultrastructural alterations were found that could be regarded as pathognomonic for one or the other form. Although in lichen amyloidosus alterations on keratinocytes seemed to be more pronounced than in macular pigmented amyloidosis, which, in contrast, shows stronger alterations of the basal membrane and the pigmentary system, these findings do not satisfactorily elucidate the histological and clinical differences between these most frequently occurring representatives of primary cutaneous amyloidosis.

Adult↗
PubMed1978

Experimental amyloidosis in mice of different ages. Effects of neonatal thymectomy and amyloidogenic stimulation of pregnant mice.

Murine amyloidosis, induced by repeated injections of sodium caseinate, was compared in young and adult Swiss albino mice and in young thymectomized and nonthymectomized mice. Thymectomized mice developed amyloidosis earlier and more severely than intact mice. Mothers of young mice were injected with sodium caseinate during pregnancy and after birth sodium caseinate injections were given to the offspring, but this treatment did not seem to induce amyloidosis in the young mice. A much shorter latent period before development of amyloidosis was seen in the adult group than in the young mice. This may be the result of a depletion of cellular and humoral immunity with aging. The shorter latent period and more severe development of amyloidosis in the thymectomized groups supports the view that an impaired immunological state may constitute a basis for the development of amyloidosis.

Aging↗
PubMed1980

[Amyloidosis. II. Current pathogenetic and clinico-nosographic aspects].

Amyloidosis is due to the overproduction of a precursor protein and its conversion into products capable of polymerisation into fibrils. The primary form, or that associated with multiple myeloma or Waldenström's macroglobulinaemia, marked by the presence of protein AL, includes the overproduction of Ig light chains followed by conversion on the part of lysosome enzymes. The forms can thus be classified as immunoproliferative diseases (plasma-cell dyscrasias). Secondary amyloidosis is primarily associated with neoplasia or chronic inflammation. Its biochemical label is the AA protein. Initially, there is overproduction of protein SAA, while the formation of AA-amyloid fibrils may theoretically be attributed to a variety of factors: changes in the amount of circulating SAA, the presence of amyloidogenetic SAA, variations in the activity of SAA catabolic systems, insufficient removal of acculated fibrils. The way in which the immunocompetent system intervenes is a controversial subject. Lesser froms of amyloidosis are known in which there are local deposits of amyloid: APUD-amyloidosis, amyloidomas. In some forms, the features of systemic accumulation are maintained, but only one organ is primarily involved. They are rarely of clinical significance (e.g. senile amyloidosis). Current biochemical techniques enable a clinical and nosographic distinction to be drawn between an Ig (AL)-amyloidosis and non-Ig (AA, APUD and AS) forms.

Amyloid↗
PubMed1993

[Familial cutaneous amyloidosis].

Familial diseases with skin lesions of amyloidosis are numerous and diverse, including widely different entities. The most homogeneous group is the one where cutaneous amyloidosis is clinically isolated and of the lichenoid type. It is probable that in most of these forms the amyloid protein is a keratin. A genetic approach of the candidate gene type would confirm or infirm this hypothesis. In the second group of diseases the lesions of amyloidosis are associated with other genodermatoses and with two other familial diseases: Partington's disease and hereditary multiple endocrine neoplasia with cutaneous and visceral lesions. The position of amyloidosis in these different diseases varies and the mechanisms of its occurrence are unknown. The third group is that of skin amyloidosis as part of hereditary systemic amyloidosis. Future advances in this matter will rest on the characterization of the amyloid protein involved and on the discovery of genetic abnormalities responsible for these diseases.

Amyloidosis↗
PubMed1996

Primary amyloidosis of the bladder: a case report.

An otherwise healthy elderly lady who presented with gross haematuria was found to have a papillary lesion in her bladder. Histological examination of the lesion showed amyloid deposits. Investigations for systemic amyloidosis were all negative. Primary amyloidosis of the bladder is a rare clinicopathological condition with only 57 documented cases in the medical literature. It often mimics bladder carcinoma in presentation and cystoscopic appearance. Amyloid deposits in the bladder may be primary (organ-limited amyloidosis) or part of systemic amyloidosis. Exclusion of systemic amyloidosis is important for management and prognosis. Localised primary lesions are best treated with transurethral resection and recurrences are uncommon. Diffuse involvement of the bladder is difficult to manage and may require urinary diversion. We report the first case of primary amyloidosis of the bladder in Singapore.

Amyloidosis↗
PubMed1996

[Histopathological study of valvular deposits of amyloid protein in cardiac amyloidosis].

Cardiac amyloidosis is associated with amyloid deposits in cardiac valves which also show the thickening of leaflets and cusps. This study examined amyloid deposits on cardiac valves to investigate the possible involvement in echocardiographic valvular abnormalities in 17 patients with systemic amyloidosis (12 males and 5 females aged 44 to 82, mean 66.4 years) in the autopsy files of the National Cardiovascular Center between 1980 and 1993. All four cardiac valves were examined histologically using hematoxylin-eosin and Congo red stains with polarization. All cusps and leaflets were divided into six segments and all segments of each cusp and leaflet were scored for the proportional area of amyloid deposit (from 0 to 3). The immunohistochemical types of amyloid proteins were immunoglobulin light chain-related (AL) amyloidosis in 16 cases, and amyloid A-related (AA) amyloidosis in one case. Twelve of 16 cases with AL amyloidosis were subclassified as AL lambda type and 4 were subclassified as AL kappa type. In the atrioventricular valve leaflets, the atrial side of basal portion showed the most remarkable amyloid deposits among the six segments. In the semilunar valves, amyloid deposits were mild in the tip and middle portions. Among the patients with AL amyloidosis, those with AL lambda type amyloid appeared to have greater deposits than those with AL kappa type amyloid. Mitral valves appearing abnormal by echocardiography had greater amyloid deposits. However, considering other factors affecting valvular function, the relationship between the localization or the degree of amyloid deposition and endocardiographic valvular abnormalities was unclear.

Adult↗
PubMed1997

[Heart failure and arterial hypertension disclosing amyloidosis].

Amyloidosis results from protein infiltration of the extracellular space of organs and tissues. Several amyloidosis proteins have been identified. Protein AL, (deriving from immunoglobulin light chain), protein AA and prealbumin are the most involved in this disease. When AL amyloidosis involves the heart, the illness is often terminal. Most clinical symptoms are heart failure and arrhythmia or block conduction. This case was characterised by the unusual combination of hypertension and amyloidosis. The diagnosis suggested by the echocardiographic but was confirmed by the damaged organ's biopsy. The present case concerns a young woman, who has hypertension and a pulmonary oedema. The echocardiographic scan showed a septal hypertrophy with a shining and granite-like aspect which is compatible with heart amyloidosis. Systolic and diastolic disorder with mitral and aortic regurgitation were also revealed. The kidney and rectum biopsies confirmed amyloidosis AL of the Kappa dysglobulinemia type, without extraosseous plasmocytoma. The heart and kidney failure symptoms disappeared after treatment with diuretics and ACE inhibitors.

Adult↗
PubMed1997

[Can amyloidosis regress?].

Amyloidosis always worsens in the absence of treatment. Suitable treatments may improve the prognosis, but results depend on the type of amyloidosis. AA amyloidosis can improve according to clinical and biological criteria after the treatment of underlying disease, or after colchicine therapy in familial mediterranean fever. Histological regression is very unusual. A small clinical improvement or at least a stabilisation can be observed in familial amyloidosis with mutation in plasma transthyretin, after liver transplantation. However, the follow-up is short and the mortality is high. In AL amyloidosis, the survival is usually less than 15 months. Some patients have a better survival when they receive chemotherapy similar to that given in multiple myeloma. This could indicate an amyloidosis improvement, or at least a stabilisation.

Amyloidosis↗
PubMed1997

[Myocardial scintigraphic studies with 123I-MIBG, 201Tl and 99mTc-PYP in patients with cardiac amyloidosis].

Myocardial scintigraphic studies, using 123I-metaiodobenzylguanidine (MIBG), 99mTc-pyrophosphate (PYP) and 201Tl were performed in 4 patients with cardiac amyloidosis. In MIBG myocardial images, 2 patients with familial amyloid polyneuropathy (FAP) showed complete or partial defect and the other 2 with primary amyloidosis had normal myocardial uptake of MIBG. In PYP myocardial images, diffuse myocardial uptake of PYP was mild in 2 patients with FAP and moderate in the other 2. 201Tl myocardial images revealed normal myocardial uptake of 201Tl in 2 patients with FAP and 1 with primary amyloidosis, and intense myocardial uptake in the other one with primary amyloidosis. These results suggest that myocardial scintigraphies with PYP and 201Tl may be useful for the detection of cardiac amyloidosis and estimation of its pathophysiology. And MIBG myocardial scintigraphy may provide useful information about sympathetic nerve abnormalities which vary with type of the fibril protein, clinical syndromes and disease process of cardiac amyloidosis.

3-Iodobenzylguanidine↗
PubMed1978

[Cardiac amyloidosis: clinical, x-ray, electric, hemodynamic, developmental and pathological aspects. Apropos of 85 cases collected from French cardiology departments].

85 cases of cardiac amyloidosis have been collected from the university cardiac departments of France. Four distinct clinical pictures have emerged: 1. Primary cardiac amyloidosis (36 cases) which combines: congestive cardiac failure, ECG signs (extreme axis deviation, low voltage, signs of myocardial necrosis), arrhythmias (67%), and a rapidly fatal outcome (23.2 m +/- 8.5); 2. Cardiac amyloidosis associated with a marked neuromuscular amyloidosis (8 cases), in patients of Portuguese extraction (4 out of 8), with a positive family history (6 out of 8), characterised by arrhythmias (5 out of 8), and with a better prognosis (1 death out of 8); 3. Cardiac amyloidosis associated with a dysglobulinaemia (14 cases) with a clinical picture which is almost identical with that of primary cardiac amuloidosis; 4. Senile cardiac amyloidosis, whose frequency increases with age, may sometimes be discovered at routine post mortem examination, and is characterised by atrial fibrillation (13 out of 27) and its association with anaemia, signs of inflammation, and coronary atheroma.

Aged↗
PubMed1998

[Primary ureteral amyloidosis].

OBJECTIVE: Two additional cases of primary ureteral amyloidosis are described. The current literature and clinical classification of the disease are reviewed. METHODS: Over a period of 18 months, we observed two cases of organic ureteral stenosis whose etiology was difficult to determine. The first case warranted a nephroureterectomy due to the condition of the compromised urinary tract. Analysis of the surgical specimen showed histological evidence of ureteral amyloidosis. In the second case, the previous experience allowed a preoperative diagnosis to be made, an exceptional situation that permitted conservative surgery. Characterization of the amyloid proteins was done in both cases with immunochemical study by Western Blot. RESULTS: Two patients with primary ureteral amyloidosis were surgically treated. The first case underwent complete excision of the urinary tract. The second case, who had a single kidney, was treated by conservative surgery. In both cases we identified a fragment of the lambda light chain of immunoglobulin by Western Blot characterization as previously reported in localized amyloidosis. CONCLUSIONS: Although ureteral amyloidosis is an infrequent disease, it should be seriously considered in the differential diagnosis of organic ureteral stenosis in order to avoid unnecessary excision. To our knowledge, these are the first cases of ureteral amyloidosis with immunochemical characterization of the amyloid protein.

Aged↗
PubMed2006

Seeking confidence in the diagnosis of systemic AL (Ig light-chain) amyloidosis: patients can have both monoclonal gammopathies and hereditary amyloid proteins.

Investigators in the United Kingdom have shown that hereditary amyloidosis can be misdiagnosed as Ig light-chain (AL) amyloidosis because family history is an ineffective screen, and tissue staining used to type amyloid is unreliable. Misdiagnosis of AL can lead to inappropriate use of chemotherapy and failure to diagnose a hereditary disease. Over a 3-year period we sought to determine how often both possible sources of amyloidosis occurred in the same patient. We employed an algorithm based on established data and patterns of amyloidosis in order to focus the screening effort. Of 178 consecutive patients referred for amyloidosis, 54 were screened by polymerase chain reaction techniques with primers designed to detect transthyretin, apolipoprotein AI, apolipoprotein AII, fibrinogen Aalpha, and lysozyme variants. Three patients (6% of those screened and 2% of symptomatic patients) had both a monoclonal gammopathy and a hereditary variant. These results justify further study of screening for hereditary variants in patients with apparent AL, and highlight the need for practical techniques for identifying fibrils extracted from tissue.

Adult↗
PubMed2006

Dermatologic adverse effects of lenalidomide therapy for amyloidosis and multiple myeloma.

OBJECTIVES: To examine dermatologic adverse effects of lenalidomide in patients with amyloidosis and multiple myeloma and to determine whether the adverse effects are different when lenalidomide is used alone compared with when it is used in combination with dexamethasone. DESIGN: Retrospective review of medical records. SETTING: Tertiary referral center. PATIENTS: Seventy-five patients with multiple myeloma and 23 patients with amyloidosis participating in clinical trials. INTERVENTION: In the 75 patients with multiple myeloma, lenalidomide was the treatment in 24 and lenalidomide and dexamethasone in 51. In the 23 patients with amyloidosis, lenalidomide was used alone. MAIN OUTCOME MEASURES: The frequency, type, severity, and time of onset of all skin eruptions that were temporally related to lenalidomide treatment were recorded. RESULTS: In the patients with amyloidosis treated with lenalidomide, 10 (43%) had rashes. In the patients with multiple myeloma, rashes occurred in 7 (29%) of those receiving lenalidomide alone and in 15 (29%) of those receiving lenalidomide and dexamethasone. The rashes were characterized as morbilliform, urticarial, dermatitic, acneiform, and undefined. Severe rashes required permanent discontinuation of lenalidomide therapy in 2 patients. In 23 patients (72%), rashes occurred in the first month after therapy was initiated; however, delayed-onset rashes occurred in 9 (28%). CONCLUSIONS: The prevalence of dermatologic adverse effects in patients receiving lenalidomide was higher in those with amyloidosis than in those with multiple myeloma. The prevalence of skin eruptions was not diminished by the concurrent use of systemic corticosteroids. Most skin eruptions were mild and did not necessitate withdrawal of lenalidomide therapy.

Amyloidosis↗
PubMed2006

Successful reduced intensity allogeneic stem cell transplantation for systemic AL amyloidosis.

No established treatments for systemic AL amyloidosis have been determined, and only four reports have described allogeneic stem cell transplantation for this disease. We report the case of a patient with orthostatic hypotension, diarrhea, nephrotic syndrome, and cardiac amyloidosis due to systemic AL amyloidosis. Reduced intensity allogeneic stem cell transplantation (RIST) was performed using a conditioning regimen comprising fludarabine 125 mg/m2 and melphalan 90 mg/m2. Hematologically complete remission and symptomatic improvement were obtained without severe transplantation-related complications. RIST may thus offer a useful treatment strategy for systemic AL amyloidosis complicated by cardiac amyloidosis.

Adult↗
PubMed1989

Elevated urokinase-type plasminogen activator level and bleeding in amyloidosis: case report and literature review.

Hyperfibrinolytic states are reported to be a cause of bleeding in patients with amyloidosis. We reviewed the literature on excessive fibrinolysis in association with amyloidosis and report our findings from a patient with idiopathic amyloidosis who developed a bleeding diathesis. Coagulation laboratory studies indicated elevated plasminogen activator levels associated with a reduction of plasminogen and alpha 2-plasmin inhibitor (alpha 2-PI) levels. The level of tissue-type plasminogen activator (t-PA) inhibitor and t-PA antigen were normal. However, the patient did have a five- to sevenfold increase in amidolytic activity for the urokinase substrate pyro-Glu-Gly-Arg-pNA (S-2444). This case therefore represents a novel example of a hyperfibrinolytic state associated with amyloidosis caused by elevated urokinase-type plasminogen activator (u-PA). Epsilon-amino caproic acid (EACA) therapy resulted in an increase in alpha 2-PI and plasminogen levels and effectively reduced the blood loss. Hyperfibrinolytic states in amyloidosis have now been reported to be due to elevated t-PA and u-PA and depleted t-PA inhibitor.

Aminocaproic Acid↗
PubMedDate not supplied

The amyloidosis of juvenile rheumatoid arthritis--comparative studies in Polish and American children. I. Levels of serum SAA protein.

Serum SAA concentration was determined by radioimmunoassay in 21 Polish children with amyloidosis secondary to juvenile rheumatoid arthritis (JRA). The results were compared to controls and children with JRA in Polish populations (where amyloidosis is a frequent complication of JRA) as well as to American children with JRA (where amyloidosis in JRA has been observed only sporadically) and American control children. No significant differences of SAA protein levels were found in the amyloidotic Polish children when compared to JRA Polish and American children. However, significantly higher levels of SAA protein were present in amyloidotic Polish children when compared to the control Polish and American group. High serum SAA protein concentration in JRA children did not necessarily correlate with the presence of secondary amyloidosis. Other mechanisms are probably involved in the development of amyloidosis.

Adolescent↗
PubMed1983

Prealbumin and retinol binding protein serum concentrations in the Indiana type hereditary amyloidosis.

Serum prealbumin and retinol binding protein (RBP) concentrations were determined for 68 members of a kindred in Indiana with a familial type of systemic amyloidosis. Immunohistochemical studies on rectal and muscle biopsy material from individuals with this type of amyloidosis revealed staining of amyloid deposits with anti-prealbumin. Both the serum prealbumin and RBP concentrations were significantly depressed in 9 patients with amyloidosis when compared with normal controls and unaffected kin. In addition, the mean RBP serum concentration of 21 offspring of the patients with amyloidosis was significantly depressed. A more significant finding was that on the basis of the serum RBP concentrations, the offspring could be divided into 2 distinct groups. One group represented approximately 50% of the children and had serum prealbumin and RBP concentrations not significantly different from their afflicted parents. The second group had serum prealbumin and RBP concentrations not significantly different from those of normal controls and non-affected kin. These findings show that prealbumin and RBP serum concentrations are depressed in patients with the Indiana type of hereditary amyloidosis and that these serum abnormalities may be present long before development of clinical disease. They suggest that individuals with this genetic abnormality may be identified prior to clinical expression of the disease.

Adolescent↗
PubMed1994

Combined treatment with terbutaline and aminophylline inhibits experimental amyloidosis in mice.

OBJECTIVE: To investigate the effects of drugs known to elevate adenosine 3':5'-cyclic monophosphate (cAMP) on experimental amyloidosis. METHODS: A beta 2-agonist, terbutaline, and a phosphodiesterase inhibitor, aminophylline, were administered in combination in a mouse model of amyloidosis induced by inflammatory stimulation with silver nitrate. Amyloidosis was quantitated by radioimmunoassay for splenic amyloid A (AA) protein. RESULTS: At the doses selected, aminophylline/terbutaline inhibited splenic amyloid deposition more potently than did colchicine, a known inhibitor of amyloidosis. CONCLUSION: Drugs known to elevate cAMP inhibit experimental mouse AA amyloidosis.

Adrenergic beta-Agonists↗