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PubMed2002

Primary localized amyloidosis of the bladder: experience with dimethyl sulfoxide therapy.

PURPOSE: We present our long-term experience with intravesical dimethyl sulfoxide (DMSO) for primary localized amyloidosis of the bladder. MATERIALS AND METHODS: The study included 4 males and 2 females 28 to 68 years old (mean age 54) at diagnosis of biopsy proven primary localized amyloidosis involving the bladder diffusely or extensively in 1 locale. All patients had normal upper urinary tracts. They continued to be symptomatic (hematuria in 3, irritative voiding symptoms in 1, and hematuria and irritative voiding symptoms in 2) despite conventional transurethral destructive therapy. Every 2 weeks they received 30-minute instillations of 50 ml. 50% DMSO intravesically for 3 months (patient 1), 6 months (1) and 1 year (4). RESULTS: Therapy failed at 3 and 6 months in 2 patients of whom 1 with a contracted bladder underwent cystectomy and another was stabilized for 1 year with laser therapy. In the remaining 4 patients who were followed for 6 years disease stabilized for 2 to 6 years (mean 3.5) but 3 later required additional therapy including repeat DMSO in 1 and laser therapy in 2. CONCLUSIONS: Diffuse or locally extensive bladder involvement by primary localized amyloidosis usually fails to respond to conventional transurethral destructive surgical procedures. Collectively, our experience and the literature suggest that intravesical DMSO can be a bladder saving measure and help resolve ureterovesical obstruction in some patients. High recurrence rate mandates lifelong cystoscopic surveillance.

Administration, Intravesical↗
PubMed1989

Xanthogranulomatous pyelonephritis and amyloidosis: a rare association.

The coincidence of systemic amyloidosis and xanthogranulomatous pyelonephritis has been reported previously only once. Clinical findings, such as the nephrotic syndrome, cardiac and autonomic nervous system dysfunction, and adrenal insufficiency, are suggestive and a thorough investigation to rule out other causes of secondary amyloidosis is warranted. We report a case of xanthogranulomatous pyelonephritis associated with secondary systemic amyloidosis and the nephrotic syndrome. Treatment consisted of nephrectomy and intensive supportive care. The unique clinical, radiographic and pathological aspects of this case are discussed.

Amyloidosis↗
PubMed1982

Renal autotransplantation for localized amyloidosis of the ureter.

Localized amyloidosis of the ureter is a relatively rare disease, causing at times ureteral stenosis with hydronephrosis and renal function impairment to various extent. Although it is not malignant nearly all reported cases have been treated by nephroureterectomy because it is clinically difficult to differentiate this entity from ureteral malignancy. We report a case in which the ipsilateral kidney was salvaged successfully by renal autotransplantation. To the best of our knowledge, this is the first case reported of renal autotransplantation for localized amyloidosis of the ureter in the English literature. We suggest that renal autotransplantation be considered in cases of localized ureteral amyloidosis.

Amyloidosis↗
PubMed2003

Clinicopathological features in ovine AA amyloidosis.

This paper describes the clinicopathological findings in sheep with AA amyloidosis. Serum samples from 12 AA amyloid-affected sheep and urine samples from 5 of these ewes were analyzed. In sera, the most important alteration was reflected in hypoalbuminemia, high concentration in beta and gamma-globulins and high levels of serum BUN, phosphorous and potassium. Serum creatinine, cholesterol and calcium concentrations showed no alterations. Urinary analysis showed proteinuria and a high protein/creatinine ratio. In three urine samples, high activities of urinary enzymes gamma-glutamyl transferase (GGT), beta-glucuronidase (GRS) and N-acetyl-beta-D-glucosaminidase (NAG) were observed, their ratios with urinary creatinine being increased for GGT and NAG and decreased for GRS. In conclusion, important alterations in biochemical and urinary parameters were observed in ovine affected by systemic AA amyloidosis. Those related to the activities of urinary enzymes could constitute reliable parameters for assessing renal injury in ovine AA amyloidosis.

Acetylglucosaminidase↗
PubMed1984

Membranoproliferative glomerulonephritis and amyloidosis.

The first known case of membranoproliferative glomerulonephritis co-existing with amyloidosis is reported. The question of whether the amyloidosis was secondary to the glomerulonephritis or whether primary amyloidosis co-existed with membranoproliferative glomerulonephritis is addressed.

Amyloidosis↗
PubMed2002

A rare case of painless gross hematuria: primary localized AA-type amyloidosis of the urinary bladder.

A 60-year-old Asian woman presented with a 1-year history of painless gross hematuria. She had no prior urologic history and no other medical problems. Her physical examination, urine cytology, and computed tomography scan were normal. Cystoscopy demonstrated several punctate areas of erythema within the bladder. Biopsies revealed amyloidosis, and immunohistochemical staining of the specimens defined the process as amyloid AA (often called secondary amyloidosis). The workup for systemic conditions associated with amyloid AA was negative. This represents an unusual case of primary localized AA-type amyloidosis of the bladder.

Amyloidosis↗
PubMed2002

Long-term recurrence of primary amyloidosis of the bladder.

An 84-year-old man presented to our department with a recurrence of a primary amyloidosis of the bladder 14 years after the first diagnosis. Follow-up had been stopped 5 years after the first procedure because no cystoscopic anomalies were noted. General evaluation confirmed the diagnosis of primary amyloidosis. The lesions were endoscopically resected. At the 3-year follow-up, the patient was free of symptoms and had remained cystoscopically free of disease. However, stable amyloidosis deposits have always been noted on the computed tomography scans. This case emphasizes the necessity of a longer follow-up, including computed tomography of the pelvis, during the 10 to 15 years after the first occurrence of the disease.

Aged↗
PubMed2002

Primary testicular amyloidosis mimicking tumor in a cryptorchid testis.

We present a case of primary testicular amyloidosis mimicking tumor in a cryptorchid testis. Examination revealed the absence of a palpable testis in the left hemiscrotum. The laboratory evaluation showed azoospermia and a low testosterone level. The radiologic investigation demonstrated a heterogeneous left inguinal mass suspicious for tumor in a cryptorchid testis. After radical orchiectomy, the histologic evaluation revealed primary amyloidosis of the testis. The pathogenesis and clinical presentation of testicular amyloidosis are reviewed.

Amyloidosis↗
PubMed1982

Serum amyloid-A protein concentration in inflammatory diseases and its relationship to the incidence of reactive systemic amyloidosis.

Serum amyloid-A protein (SAA) is the putative precursor of amyloid-A (AA) protein which forms the fibrils in reactive systemic or secondary amyloidosis. By means of a novel immunoradiometric assay, the concentration of SAA was found to be greatly elevated in patients with rheumatoid arthritis and juvenile chronic arthritis and correlated with activity of their primary disease. However, in patients with systemic lupus erythematosus SAA levels were only modestly raised, even in those with severe active disease, unless significant intercurrent microbial infection was also present. In Crohn's disease SAA levels showed a pattern similar to that seen in rheumatoid arthritis, whereas in ulcerative colitis it resembled that of systemic lupus erythematosus. The level of SAA response in these different disorders corresponds with the incidence of reactive systemic amyloidosis in them. These observations support the view that major increases in SAA levels are a necessary condition for the deposition of this form of amyloid and suggest that prospective monitoring of the SAA concentration in predisposing diseases may help to identify those individuals who are most at risk for amyloidosis.

Amyloid↗
PubMed1982

An unusual case of ocular involvement in primary systemic nonfamilial amyloidosis.

A case of primary amyloidosis with vitreous involvement was diagnosed by study of a specimen obtained by pars plana vitrectomy. Vitreous opacities had been interpreted clinically as a vitreous hemorrhage. Medical evaluation after study of the vitreous specimen failed to demonstrate evidence of amyloidosis, although postmortem examination 19 months later established the systemic diagnosis of nonfamilial systemic primary amyloidosis. Both eyes were obtained postmortem. Amyloid involvement of the vitreous, retina, and choroid was found. There was also amyloid deposition in the trabecular meshwork, although the intraocular pressure was normal. Amyloid was deposited along the anterior, pupillary, and posterior surfaces of the iris. The histochemistry, ultrastructure, and treatment of this condition are discussed.

Amyloid↗
PubMed1991

Climatic droplet keratopathy with corneal amyloidosis.

Climatic droplet keratopathy (CDK) is a degenerative disease of the cornea, highly prevalent in Saudi Arabia. It is characterized by aggregates of small, golden-yellow globules that accumulate in the subepithelial layers, Bowman's layer, and the superficial stroma of the cornea. The authors report on 22 patients (40 eyes) with CDK and lattice lines in the cornea. The condition occurred late in life (mean age, 67 years). It was bilateral in 82% of patients, with no family history of lattice dystrophy or systemic amyloidosis. These lines were found in all layers of the stroma. Results of histopathologic and electron microscopic examination of the lattice lines confirmed the presence of amyloid. Results of immunohistochemical examination showed evidence of deposition of AP protein. The cause of these deposits in the cornea is not known, but the authors speculate that environmental factors causing CDK also may play a role in the deposition of protein AP in the cornea. The contribution of trachoma to the degenerative changes in the cornea and secondary amyloidosis could not be ruled out, as all patients had inactive trachoma. Climatic droplet keratopathy with corneal amyloidosis, therefore, represents a form of "lattice degeneration" of the cornea.

Adult↗
PubMed1993

Spheroidal keratopathy associated with subepithelial corneal amyloidosis. A clinicopathologic case report and a proposed new classification for spheroidal keratopathy.

PURPOSE: An unusual case of primary spheroidal degeneration associated with subepithelial corneal amyloidosis is described clinically and histopathologically, and a new classification for spheroidal keratopathy is proposed. METHODS: The corneal button obtained by lamellar keratoplasty was examined by light and transmission electron microscopy. Amyloid deposits were evaluated further by means of the thioflavin T fluorescence technique, the potassium permanganate reaction, and immunohistochemical studies using antibodies against immunoglobulin light chains and proteins AA and AP. RESULTS: Spheroidal droplets were observed in basement membrane, Bowman's layer, and anterior stroma, and exhibited characteristic staining with Verhoeff's iron hematoxylin. Amyloid deposits were found beneath the epithelium, almost exclusively in the central area of the corneal button. The deposits showed no immunohistochemical staining with antibodies to amyloid proteins AA and AP or kappa and lambda light chains. CONCLUSIONS: The histochemical properties of the spheroidal droplets in this case were similar to those observed by other authors and suggested a sulfur-rich protein other than keratin. The combination of clinical and pathologic findings permitted the differential diagnosis of subepithelial amyloidosis and distinguished this condition from that of gelatinous drop-like dystrophy. To the authors' knowledge, this is the first description of primary spheroidal keratopathy associated with secondary subepithelial corneal amyloidosis in an eye that had not undergone previous keratoplasty.

Adult↗
PubMed1993

Hepatic amyloidosis in intravenous drug abusers and AIDS patients.

The clinical and pathological features of four liver biopsies and 12 autopsies from 1981-1990 with hepatic amyloidosis were reviewed. All of the patients had a history of both intravenous and subcutaneous cocaine and heroin use with chronic suppurative skin ulcers. Five patients were proven to have the acquired immunodeficiency syndrome at autopsy. Systemic amyloidosis was diagnosed in only five out of the 16 patients prior to death. Hepatomegaly was present in 12 patients. The amyloid protein was AA in 14 and AL in one case. Definitive characterization of the amyloid substance was not possible in one case. There was no evidence of multiple myeloma or a plasma cell dyscrasia in the one patient with AL amyloid. The amyloid distribution in the liver was both parenchymal and vascular in 12 cases. Cholestasis, which appeared to occur preterminally, was the only histological finding that correlated with the patient's clinical condition. The histological pattern of amyloid in the liver could not predict the type of amyloid protein that was identified. Amyloidosis should be considered in the differential diagnosis of unexplained hepatomegaly in the acquired immunodeficiency syndrome with chronic suppurative infections.

Acquired Immunodeficiency Syndrome↗
PubMed1986

Systemic reactive amyloidosis caused by hepatocellular adenoma. A case report.

Systemic reactive amyloidosis caused by non-malignant tumors seems to be extremely rare. In the present paper a 39-year-old female with systemic reactive amyloidosis caused by a hepatocellular adenoma is described. The patient had a history of using contraceptive drugs. She had a pronounced nephrotic syndrome, a renal biopsy showing reactive (AA) amyloidosis, and a bone scintigraphy (99mTc methylene diphosphonate, TcMDP) showing an increased uptake in soft tissues. Removal of the adenoma was followed by a gradual resolution of the nephrotic syndrome and a normalization of the bone scintigraphy. The tumor was histologically a mixture of focal nodular hyperplasia and liver cell adenoma.

Adenoma↗
PubMed2000

Myeloma-associated systemic amyloidosis presenting as chronic paronychia and palmodigital erythematous swelling and induration of the hands.

Mucocutaneous involvement occurs predominantly in primary systemic amyloidosis as well as in myeloma-associated systemic amyloidosis. It is rarely observed in other types of amyloidoses. Signs of such involvement may aid in the early diagnosis of the disease process. Herein, we describe a 64-year-old white male patient with myeloma-associated systemic amyloidosis in whom the disease presented with unique cutaneous lesions consisting of chronic paronychia and palmodigital erythematous swelling and induration of the hands. Following weekly regimens with prednisone (20 mg/day) and melphalan (2 mg/day) administered every 16 weeks, almost complete resolution of the cutaneous lesions was observed after 1 year of therapy. Also, in response to chemotherapy, modest regression of the myelomatous bone lesions and complete resolution of the underlying gammopathy occurred.

Amyloidosis↗
PubMed1988

Primary cutaneous amyloidosis of the auricular concha.

Several patterns of primary cutaneous amyloidosis are recognized; in this report of four cases, we define a new pattern in which the lesions are composed of small papules grouped on the concha of the ear. The lesions typically are not pruritic but slightly friable. They may be present on both ears. The patients do not have lesions of amyloidosis on other parts of the body and no known insult precedes the appearance of lesions. This entity appears to have been described previously as collagenous papules of the ear. Histochemical and electron microscopic methods allow us to confirm the amyloid nature of the substance. We believe this condition to be a variant of primary cutaneous amyloidosis, particularly because monoclonal antikeratin antibody EKH4, which has been positive in lichenoid, macular, and skin epithelial tumor-associated amyloidoses, was positive in our cases.

Adult↗