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[Rare case of palatal amyloidosis].

Amyloidosis first described by Karl Rokitansky in 1842 consists in accumulation of pathological protein (amyloid) within both cells and extracellular space. It usually develops in the course of a chronic inflammation or some neoplasms. The clinical picture depends on the impairment degree of an organ in which amyloid accumulates. The diagnosis is based on the cytological examination of the material collected by means of biopsy of the subcutaneous fatty tissue and of the mucosa of the anal orifice, gums, kidneys or liver etc. In the presented study a rare case of amyloidal concrements localised in the palatal region has been described. Literature has usually presented amyloidosis localised in the tongue, lips, rhino-pharynx and larynx. Amyloidosis localised in the region of the head and neck occurs very rarely. In the presented case amyloidal deposition in any other site was not reported.

Amyloidosis↗

[Association of IGg multiple myeloma with pulmonary amyloidosis: about one case].

Pulmonary amyloidosis is a rather rare complication of multiple myeloma particularily the rare Ig D myeloma. It is often generalized and is seen in a late stage of the disease. We report a case of an Ig G myeloma complicated of a pulmonary amyloidosis in a 66-year-old man hospitalised for infectious pulmonary disease with a radiologic interstitial syndrome. Discovery of the multiple myeloma and of the amyloidosis was fortuitous.

Aged↗

Renal amyloidosis (Part II).

As reported in the previous chapter (Part I) the term amyloidosis refers to the deposition of an amorphous substance defined by the presence of fibrillary structure by electron microscopy and a characteristic betapleated sheet structure by X-ray diffraction. Renal biopsy is fundamental in diagnostic terms because morphological demonstration is necessary and the tissue most frequently involved by amyloidosis is the kidney. In the previous chapter we presented the different histochemical and immunohistochemical techniques that are necessary to differentiate the two main types of amyloidosis defined as AL and AA. Here we show the variability of deposition of amyloid in glomerular, vascular and interstitial compartments.

Amyloidosis↗

Renal cancer in a patient with Crohn's disease and secondary amyloidosis.

Herein we report the case of a patient suffering from renal amyloidosis secondary to Crohn's disease (CD) who presented with anuria and anasarca 8 years after commencement of colchicine treatment. End-stage renal disease was confirmed. Renal imaging prior to hemodialysis showed tumor of the left kidney. Histopathology after nephrectomy revealed secondary amyloidosis as well as the existence of cancer (hypernephroma). We discuss the presence of renal amyloidosis secondary to CD and at the same time renal cancer development, a combination that has not been reported so far, and we emphasize the surprisingly long renal survival of this patient after colchicine treatment.

Adult↗

[Localized nodular pulmonary amyloidosis; report of a case].

A 66-year-old female was admitted to our hospital because of chest abnormal shadow. Chest X-ray and chest computed tomography (CT) on admission showed a nodule in the right middle lobe. The nodule was not diagnosed preoperatively by a bronchoscopy. She underwent partial lung resection including the nodule with video-assisted thoracoscopic surgery. The pathological diagnosis was amyloidosis, and we diagnosed her illness as localized nodular pulmonary amyloidosis, since the amyloid substance was type AL. In addition, electron microscopy showed amyloid as straightly fibrous materials in alveolus. It is difficult to differentiate amyloidosis from lung cancer by radiology, and the lung biopsy with video-assisted thoracoscopic surgery is useful and a safety way to establish diagnosis.

Aged↗

[Crohn's disease and amyloidosis. An association to be considered].

Two instances of Crohn's disease and amyloidosis are presented. The time elapsed between the diagnosis of Crohn's disease and that of amyloidosis was 2 and 8 years respectively. The first case had hepatosplenomegaly and moderate proteinuria; the other one, severe proteinuria in the nephrotic syndrome range. One patient had ileocolic disease and the other disease limited to the terminal ileum. In both cases renal functional was normal. The diagnosis was made by liver biopsy in one patient and by repeated renal biopsy in the one with nephrotic syndrome. Ileocolic resection was undertaken in one patient without complications. Screening for amyloidosis is important in patients with Crohn's disease.

Adult↗

[Secondary bladder amyloidosis: a case of salvage cystectomy for massive hematuria].

Amyloidosis of the bladder associated with systemic amyloidosis secondary to rheumatoid arthritis is a rare disease. Due to the absence of routine screening, its incidence appears to be understimated and only 11 cases, all symptomatic, have been reported in the literature. The symptoms of this disease are dominated by macroscopic haematuria, always massive, which may be life-threatening. We report a case in which the severity of this haematuria required emergency cystectomy to ensure haemostatis, which revealed amyloidosis on histological examination.

Amyloidosis↗

Portal hypertension and intrahepatic cholestasis in hepatic amyloidosis.

Despite the fact that hepatic involvement is frequently seen in systemic amyloidosis, major clinical symptoms or impaired hepatic functional capacity are rare. We describe a patient with primary hepatic amyloidosis, severe intrahepatic cholestasis and portal hypertension, a combination previously reported only three times in the literature. In case of an unexplained intrahepatic cholestasis or portal hypertension the possibility of amyloidosis should be considered and a Congo red staining should be performed.

Aged↗

Amyloidosis of the oral cavity: report of five cases.

Five cases of amyloidosis involving structures in the oral cavity are reported. Three cases appeared to be secondary systemic amyloidosis, one case was the systemic form associated with multiple myeloma, and one case appeared to represent nodular or localized amyloidosis. All cases appeared in persons over 63 years of age, and the most common area of involvement was the tongue. The nature of so-called amyloid deposits in certain odontogenic tumors is discussed in light of recent knowledge of amelogenins and enamelins.

Aged↗

Perforation and tumor formation of the intestine in primary amyloidosis.

We report a case of primary amyloidosis with repeated bowel perforations. This patient also had localized amyloid deposition creating a tumorous region mimicking malignancy in the rectum. Perforation of the intestine is common in systemic amyloidosis. The ischemic change due to amyloid infiltration into the vessel wall may lead to perforation of the affected bowel. Amyloid tumors occur more often in localized amyloid than in systemic amyloidosis. Macroscopically, it is difficult to distinguish amyloid tumor of the intestine from neoplasia.

Amyloidosis↗

Amyloidosis in a case of ankylosing spondylitis with a review of the literature.

A 53-year-old man with a 20-year history of ankylosing spondylitis presented with proteinuria and acute renal insufficiency. Amyloidosis was diagnosed on renal biopsy. The association of secondary amyloidosis has been described with collagen vascular diseases, however, a review of the literature reveals few cases associated with the spondyloarthropathies. We were able to find only 2 cases in the American literature. Thus, although a rare complication, amyloidosis should be considered in the differential diagnosis of proteinuria and renal insufficiency in spondyloarthropathy.

Amyloid↗

[Renal involvement in amyloidosis: analysis of 14 cases].

UNLABELLED: 14 cases were investigated between 1963 to 1988. Age ranging from 25-66 (mean 48.2). 9 cases (64%) were over age 50. Diagnosis was established by renal biopsy or autopsy. 11 cases (79%) were primary. 3 cases (21%) were secondary with chronic infection. Besides renal involvement, primary amyloidosis principally affected the heart, gastrointestinal tract and tongue. Hepatosplenomegaly and anemia were found in secondary cases. Proteinuria was found in 14 cases. Nephrotic Syndrome (NS) found in 8 cases, both renal insufficiency and hypertension were 21% respectively. In 3 cases performed renal venography, 2 cases showed renal vein thrombosis. 9 cases (64%) showed kidneys were enlarged. Congo red test showed positive reaction were 100% in secondary and 50% in primary. By light microscopy stained with congo red there was a specific protein deposited in glomeruli and mesangium, by electron microscopy. There was fine amyloid fibrils. These findings are characteristic and to have diagnostic value. CONCLUSION: (1) Renal amyloidosis is not very rare in China. (2) Proteinuria and NS were major clinical manifestations. (3) Renal biopsy and autopsy have diagnostic value, congo red test does not prove the absence of primary amyloidosis.

Adult↗

[Nodular amyloidosis of the lungs associated with Sjögren's syndrome].

Pulmonary amyloidosis can develop together with both primary or secondary amyloidosis, isolated or associated with amyloidosis of other organs. In the study a case of a 59 year old female with multiple nodules and diffuse amyloid infiltration in pulmonary parenchyma associated with Sjörgen's syndrome is presented. Amyloid deposits were also found in the surgically removed parotid salivary gland, mostly in its blood vessel walls.

Amyloidosis↗

[Amyloidosis maculosa: diagnosis in primary care].

Amyloidosis maculosa is a clinical entity with low incidence factor in our medium, which basically affects middle-aged women. The lesion is characterised by the presence of poorly defined, hyperpigmented, brownish or greyish maculae that converge and focus basically on the upper back and shoulders, usually accompanied by pruritus. Three patients were erroneously catalogued for years as having pityriasis versicolor. Two of these patients presented a typical clinical amyloidosis maculosa, and the third presented a less common manifestation of the disease: a single, well-defined lesion in the subscapular region. We believe that the approach to the diagnosis of pityriasis versicolor with hyperpigmented lesions that do not respond to specific treatment should be revised. Although amyloidosis maculosa has a low incidence in our medium, it is an entity which should not be discarded in these cases.

Adult↗

Amyloid in familial amyloidosis, Finnish type, is antigenically and structurally related to gelsolin.

Immunohistochemical studies of six patients with familial amyloidosis, Finnish type, showed that their amyloid deposits did not react with polyclonal antibodies against the amyloid proteins of other, established forms of systemic or cerebral amyloidosis. However, strong immunoreactivity was observed with rabbit antiserum raised against a low molecular weight purified amyloid subunit isolated from one of the patients. This immunoreactivity was abolished by absorption with the low molecular weight amyloid fraction. The amino terminal sequence of the amyloid protein subunit was homologous to gelsolin, an actin-modulating protein, and the amyloid deposits in tissues reacted with a monoclonal antibody against gelsolin. These studies show that the amyloid protein in familial amyloidosis, Finnish type, is not related to previously identified forms of amyloid, including prealbumin (transthyretin) variants, but represents a novel amyloidogenic protein related to gelsolin, a plasma and cytoplasmic protein.

Actins↗

[Unusual primary cutaneous localized amyloidosis].

The case of a 41-year-old female patient with an unusual type of primary localized cutaneous amyloidosis is reported. Clinical examination revealed lesions typical for macular cutaneous amyloidosis, while histology and histochemistry, in contrast, revealed the presence of nodular amyloidosis of the skin with deposition of lambda light chain amyloid.

Adult↗

Amyloidosis in saphenous vein aortocoronary bypass grafts.

A patient in whom idiopathic amyloidosis of aortocoronary saphenous vein grafts was found at autopsy two years after myocardial revascularization due to coronary atherosclerosis is reported. Idiopathic generalized immunocyte derived amyloidosis extensively studied at autopsy was obviously present at the time of surgery although it remained unnoticed macroscopically in the inserted graft. It appears that simultaneously with arterialization further deposition and also significant redistribution of amyloid within the walls of the vein grafts additionally took place after their insertion. It seems interesting that in spite of the amyloidosis the grafts functioned well and were found patent two years after surgery.

Amyloidosis↗

[The chemotactic characteristics of the leukocytes and fibroblasts in amyloidosis].

Altogether 44 patients with amyloidosis (7 with primary, 24 with secondary and 13 with hereditary associated with periodic disease) and different stages of renal impairment and 32 patients with chronic glomerulonephritis (CGN) (14 with the latent and 18 with the nephrotic form) were examined for chemotactic properties of peripheral blood leukocytes and skin fibroblasts in Boyden's chambers. Chemotaxis of leukocytes from patients with different clinical forms of amyloidosis was found to be decreased. That decrease manifested itself even at the proteinuric stage of renal impairment. Addition of autologous serum brought about another decrease of the chemotactic response of leukocytes. There was a lowering of chemotaxis of cultivated skin fibroblasts from amyloidosis patients, which remained unchanged on repeated cell passages. Chemotactic activity of cultivated fibroblasts from CGN patients did not differ from that of healthy persons' fibroblasts and embryonal fibroblast strain. Possible causes of the alterations revealed are discussed.

Adolescent↗