Localized amyloidosis of urinary bladder: a case report

A case of localized amyloidosis of the urinary bladder is reported. A 82-year-old woman visited our hospital with the chief complaint of miction pain and residual urine sensation. Cystoscopic examination revealed a broad-based and nonpapillary tumor without bleeding on the right lateral wall. A transurethral biopsy of this tumor was performed. A histopathological examination with H.E. and Congo red stains demonstrated amyloid deposition in the submucosal layers of the vesical wall. Rectal biopsy and other findings suggested no deposition of amyloid in other organs. On the basis of these findings, we made a diagnosis of localized amyloidosis of the urinary bladder. To the best of our knowledge, the present case is the 23rd of localized amyloidosis of urinary bladder in Japan. The patient was asymptomatic after biopsy. We discuss the clinical features and management of this disease.     

Resolution of nephrotic syndrome and lack of progression of heroin-associated renal amyloidosis

The case of a patient with nephrotic syndrome and renal insufficiency due to renal amyloidosis secondary to chronic subcutaneous heroin abuse who discontinued her drug habit is reported. During the 6 years following the initial renal biopsy, the patient's nephrotic syndrome remitted: urinary protein decreased from 6.8 g/d to 170 mg/d, serum albumin increased from 2.5 g/dL to 3.9 g/dL, and she was no longer edematous. Serum creatinine remained stable at 1.8 mg/dL and creatinine clearance was also unchanged at 34 mL/min. Repeat renal biopsy showed AA amyloidosis involving the glomeruli, with minimal interstitial inflammation. This appears to be the first case of clinical improvement in heroin-associated renal amyloidosis after cessation of drug injections. As in other cases of AA amyloidosis that have improved, the clinical picture does not correlate with the findings on renal biopsy.     

Primary localized amiloidosis with recurrent relapse: a case report]

This is a case report of primary localized amyloidosis of the urinary bladder. The patient was a 73-year-old woman who had relapse of this disease three times. Primary localized amyloidosis of the urinary bladder is an especially rare disease and the prognosis is excellent in most cases. We performed transurethral resection of the bladder tumor (TUR-Bt) each time and we are following up this patient carefully. After the last TUR-Bt, she has had no recurrence for 7 months. Considering multifocal recurrence and excellent prognosis, we stress the importance of TUR-Bt for diagnosis and careful follow up.     

Localized amyloidosis of the ureter and bladder treated effectively by occlusive dressing technique therapy using dimethyl sulfoxide: a case report

We report here the first case of localized amyloidosis of the ureter and bladder to be treated effectively by occlusive dressing technique therapy using dimethyl sulfoxide. The patient was a 48-year-old woman whose chief complaint was macrohematuria and right back pain. Ultrasound sonography demonstrated right hydronephrosis and an intravesical mass in the region of the right ureteral orifice. Retrograde pyelography revealed severe stricture of the right lower ureter. Cystoscopy demonstrated a yellow submucosal tumor around the right ureteral orifice. We suspected urinary tract amyloidosis, and transurethral biopsy and resection of the intravesical mass were performed under right ureteral stenting. Histopathological diagnosis was amyloidosis. There was no evidence of systemic amyloidosis. To treat residual amyloidosis of the ureter and bladder, we performed occlusive dressing technique therapy using dimethyl sulfoxide every day. After 6 months of therapy, the right hydronephrosis disappeared, and there was no evidence of a recurrence of amyloidosis. We concluded that this therapy was very effective and safe for urinary tract amyloidosis.     

A Case of Primary Localized Amyloidosis of the Penile Urethra

Primary localized amyloidosis of the penile urethra is rare. We report a case in which the urethra was obstructed by amyloid tissue without evidence of systemic amyloidosis. After urethral dilatation, the patient reported a markedly improved urinary stream with minimal dysuria.     

Lokalisierte Amyloidose des Harnleiters

Localized amyloidosis of the ureter is a rare condition. Because of the difficulty in differentiating between localized amyloidosis and an obstruction due to other benign or malignant conditions of the urinary tract, in some cases even an unnecessary nephroureterectomy is performed. We describe a patient with obstructive amyloidosis of the right ureter. Diagnosis was confirmed by endoscopy with biopsies. The patient was treated successfully by partial ureterectomy and ureteroneocystostomy. No systemic involvement of other organs was detected and after a 2-year follow-up no local recurrence developed.     

Amyloidosis of renal pelvis and urinary bladder.

We describe a patient with amyloidosis of renal pelvis and 2 patients with urinary bladder amyloidosis. Clinical presentation in all of the cases mimicked cancer of the respective sites. Clinical diagnosis of amyloidosis is not possible, making biopsy mandatory. Immunology of amyloid formation and treatment of amyloidosis are discussed.     

Primary AA type amyloidosis of the urinary bladder: a case report

Primary amyloidosis of the urinary bladder is a rare disease entity. A total of 61 cases have been reported in the Japanese literature, and most of them were AL type amyloidosis. We report here a case of primary AA type amyloidosis. A 52-year-old man presented with a chief complaint of asymptomatic gross hematuria. Cystoscopy revealed yellowish elevated lesions, transurethral mucosal biopsies were performed, and the histopathological diagnosis indicated a primary AA type amyloidosis of the urinary bladder. Systemic amyloidosis was clinically eliminated. The yellowish lesions in the bladder through cystoscopy disappeared spontaneously one year later without any specific treatment, but periodical work-up may be necessary to rule out recurrence of the disease or bladder tumor.     

A case of primary diffuse tracheobronchial amyloidosis

Tracheobronchial amyloidosis (TBA) is an uncommon localized form of amyloidosis with fewer than 150 reported cases in the literature. We report a case of primary diffuse tracheobronchial amyloidosis who presented with cough, wheezing, recurrent and progressive dyspnea as well as hemoptysis. Though there is no universally accepted treatment for the localized form, this patient was successfully treated with three sessions of bronchoscopic resection and adjuvant steroids. This report will review the various types of amyloidosis of the respiratory tract and their clinical features, in addition to discussing the different available treatment modalities for TBA.     

Resolution of primary amyloidosis by melphalan and prednisolone: a case report

We here report a case of a 50-year-old man who showed histologically evident resolution of primary amyloidosis by melphalan and prednisolone. The patient was admitted to our hospital for further evaluation of nephrotic syndrome and remarkable hepatomegaly with refractory ascites, on September 11, 1998. Laboratory tests at presentation showed nephrotic syndrome with slight renal impairment and elevation of the enzymes of the biliary system. Monoclonal light chains were not detected in the serum or urine by immunoelectrophoresis. A renal biopsy revealed global deposition of amyloid in all glomeruli, interstitium and blood vessels. Immunofluorescence staining was positive for kappa light chains. Liver biopsy specimens showed extensive deposition of amyloid along sinusoid walls. Bone marrow aspiration contained 7% plasma cells but no clusters or abnormal cells. Based on these findings, systemic AL- (amyloid light chain) amyloidosis was diagnosed, and the treatment with combinations of melphalan and prednisolone was started from October 1998 at intervals of 4-6 weeks. Renal impairment progressed, resulting in the initiation of maintenance hemodialysis in February 1999. Reinfusion of ascitic fluid into the hemodialysis circuit had been performed from March 1999 for refractory ascites, and ascites disappeared in July 1999. Furthermore, urinary output increased after 14 courses of chemotherapy. Renal function gradually ameliorated with a concomitant reduction in the enzymes of biliary system, and finally hemodialysis was discontinued in April 2001. Sixteen courses of chemotherapy were administered by April 2001. Proteinuria was negative in August 2001. A second renal biopsy was performed on November 20, 2001, which showed markedly decreased amyloid deposition and a proliferation of mesangial cells and increase in matrix in various degrees. We report a case of a patient with primary amyloidosis who was successfully treated by melphalan and prednisolone, resulting in marked resolution of renal amyloidosis.     

A case of primary systemic amyloidosis with AA protein

We present here a case of young adult-onset primary systemic amyloidosis with AA protein. A 29-year-old male presenting with macrohematuria was transferred to our hospital because of aggravation of renal function. Amyloid protein was detected in the bladder, stomach and thyroid. The amyloid protein proved to be AA type by potassium permanganate histochemical analysis. Since secondary amyloidosis was excluded by the laboratory data and there was no family history of amyloidosis, a diagnosis of primary systemic amyloidosis with AA protein was reached. Although the patient was prescribed dimethyl sulphoxide, his renal function worsened with gastrocolic symptoms and a bleeding tendency. Hemodialysis (HD) was then initiated. After starting the HD, the patient's general condition recovered and subsequently the patient on treatment with maintenance HD was discharged.     

Primary localized AA type amyloidosis of urinary bladder: case report of rare cause of episodic painless hematuria

A 45-year-old diabetic man presented with an episodic history of painless gross hematuria. He had had no previous urinary symptoms or any other medical problems. His physical examination, urine cytology, and computed tomography scan findings were normal. Cystoscopy demonstrated two nodular masses in the urinary bladder with a hemorrhagic mucosal surface. Biopsies revealed amyloidosis, and immunohistochemical staining of the specimens defined the process as amyloid AA (mostly seen in 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.     

Primary localized amyloidosis of the ureter

Primary amyloidosis is a rare condition that can involve the urinary tract. These lesions can occur anywhere in the collecting system and are often mistaken clinically for malignancies. We report a case of localized ureteral amyloidosis. Our treatment consisted of a conservative approach with local resection.     

Unresolved lytic lesions following parathyroidectomy in a patient with chronic renal failure

With advances in the treatment of patients with chronic renal failure, their life expectancy has increased. In turn, the prevalence of osteitis fibrosa cystica, a manifestation of secondary hyperparathyroidism, and beta2 microglobulin amyloidosis, a result of long-term haemodialysis, has risen. While both conditions share similar radiological features, their management is very different. We present the case of a patient with renal failure who had been receiving haemodialysis for over 20 years. Lytic lesions had been observed in the proximal part of both femurs for ten years. A presumptive diagnosis of osteitis fibrosa cystica was made. However, no regression of the lesions occurred after parathyroidectomy. The patient subsequently developed sequential pathological fractures through the lesions, for which bilateral total hip replacements were performed. Histology of the lesions revealed that the patient was in fact suffering from amyloidosis. In patients with chronic renal failure, osseous amyloidosis is a highly probable differential diagnosis, especially if no regression of a lytic lesion is observed after parathyroidectomy.     

局限性胃淀粉样变病

目的 提高局限性胃淀粉样变病的诊断率。方法 报告１例局限性胃淀粉样变病并复习有关文献,对其病因、发病机制、临床表现、诊断、治疗及预后进行分析。结果 本例局限性胃淀粉样变病患者无家族史及慢性疾病史,全胃切除术后病理诊断为局限性胃淀粉样变病ＡＡ型,其他组织如食道、十二指肠、直肠、骨髓未见淀粉样变质沉淀。结论 对于胃恶性肿瘤的鉴别诊断,应考虑本病的可能;为避免本病并发症的发生,应行切除手术。     

Localized amyloidosis of the urinary bladder: a case report

Amyloidosis is characterized by extracellular deposition of abnormal insoluble fibrils, which cause structural and functional disorders. Amyloidosis is classified into systemic and localized amyloidosis. Localized amyloidosis in individual organs is uncommon. We report a rare case of localized form of primary amyloidosis of the urinary bladder. A 76-year-old male visited our hospital with a complaint of macroscopic hematuria. Cystoscopy showed submucosal hematoma in the anterior wall and broad-based mass occupying the trigone without normal mucosa covered by calcification. Transurethral biopsy and resection were performed. Histopathological diagnosis was AL type amyloidosis occupying submucosal extracellular space. We gave the patient occlusive dressing with dimethyl sulfoxide. In 12 months, cystoscopy and magnetic resonance imaging revealed improvement of the mass-like lesion in the bladder wall.     

Sequential Liver,Kidney, and Autologous Stem Cell Transplant for AL Amyloidosis: A Case Report

AL type amyloidosis is a systemic disease characterized by the accumulation of amyloid fibrils that can affect many organs such as the skin, gastrointestinal tract, heart, lungs, liver, and kidney. The most frequently involved organ in amyloidosis is the kidney, but cardiac amyloidosis with the poor prognosis is amyloid organ involvement. In this study, we present the treatment of a 40-year-old female patient with acute Budd-Chiari syndrome and very severe proteinuria with sequential liver, kidney, and autologous stem cell transplant after the diagnosis of systemic amyloidosis. To reduce the effects of massive proteinuria and very severe hypoalbuminemia, bilateral renal artery embolization was performed first. After the evaluation of the patient, she underwent liver transplant from a deceased donor, and then kidney transplant was performed from her son 1 month later. Afterward, the patient was discharged without any problems and underwent chemotherapy and stem cell transplant for primary AL amyloidosis. She was followed up without any problem in terms of liver, kidney, and stem cell at the 24th postoperative month. This case shows that autologous stem cell transplant after kidney and liver transplant may be a good treatment option in a selected patient with stem cell involvement diagnosed as having AL amyloidosis.     

Primary amyloidosis of the bladder: a case report

A case of primary amyloidosis of the bladder, which developed 4 years after primary treatment, is described. The patient is a 55-year-old male, who consulted our clinic with a complaint of asymptomatic gross hematuria on October 1, 1983. Cystoscopic examination showed a tumorous lesion involving right lateral wall of the bladder, which was resected transurethrally. No malignancy was found by histopathological examination. About 4 years later, he consulted our clinic again with a complaint of asymptomatic gross hematuria. There was a tumorous lesion involving left lateral wall of the bladder on cystoscopy. Transurethral resection of bladder tumor was performed under a diagnosis of bladder tumor. Histological examination revealed amyloidosis of the bladder, AL-type amyloid. Re-examination of the specimens resected 4 years ago showed the same findings. Biopsies from the tongue, skin and rectum disclosed no amyloid deposition. During these 19 months, he had no recurrence. This case was thought to be the first one of primary amyloidosis of the bladder showing new recurrence reported in Japanese literature.     

Gastric amyloidosis mimicking malignancy. A case report

Amyloidosis is an abnormal extracellular deposition of insoluble proteins, which is associated with an involvement of the gastrointestinal tract in 50 to 70% of cases. In primary amyloidosis (light chain amyloidosis), localized gastric involvement is a rare finding which can mimic malignancy. We report the case of a 56-year-old man, admitted with upper digestive outlet obstruction. Linitis plastica with lymph node involvement was suspected by gastroscopy, barium meal and endoscopic ultrasonography but was not confirmed by gastric biopsies. The patient was treated with total gastrectomy with lymph node dissection. Pathological examination demonstrated gastric and lymph nodes amyloidosis and no malignant tumor was found. The patient died 9 months later from cardiac failure due to amyloidosis.     

Primary amyloidosis of lower urinary tract

Primary amyloidosis of the lower urinary tract is a rare condition with an excellent prognosis in most cases. Three patients with this condition are described. In the cases of localized amyloidosis of the urethra and urinary bladder, the clinical presentation mimicked cancer of the respective sites. This was also true in the case of primary systemic amyloidosis involving the bladder. If significant associated systemic or local disease can be excluded, management is symptomatic and expectant.