Original ArticlePrimary Localized Amyloidosis of the Urinary Bladder: A Case Series of 31 Patients
Section snippets
PATIENTS AND METHODS
We identified 31 patients with primary localized amyloidosis of the bladder who were seen at the Mayo Clinic in Rochester, Minn, between 1944 and 1996. Patients with primary systemic amyloidosis and amyloidosis secondary to rheumatic conditions, infections, and malignancies were excluded. Eight of the patients have been described previously by authors from our institution'; this article includes these 8 patients and their follow-up information as well as 23 patients examined subsequently at the
Sex and Age Distribution
The 31 patients consisted of 22 men (71%) and 9 women (29%). The median age of the 31 patients at diagnosis was 55 years (range, 28-80 years). In men, the disease was almost evenly distributed over the fifth, sixth, and seventh decades of life, whereas in most women (56%), the disease was diagnosed in the sixth decade of life (Table 1).
Symptoms
Of the 31 patients, 24 (77%) presented with gross, painless hematuria. In 6 of these patients, the hematuria was associated with irritative symptoms of the lower
DISCUSSION
Amyloidosis is characterized by deposition, in various tissues, of a homogeneous eosinophilic material that has a nonbranching branching fibrillar ultrastructure. Most cases occur in a generalized form as a manifestation of an underlying plasma cell neoplasm (myeloma) or plasmacytic dyscrasia. Systemic amyloidosis is a progressive and usually fatal disease. The amyloid fibrils in primary systemic amyloidosis are derived from monoclonal immunoglobulin light chains (AL type). Localized
CONCLUSION
Primary localized amyloidosis of the urinary bladder is a rare disorder. It is of interest to the generalist and the urologist because it can be easily confused with carcinoma. Immunohistochemical subtype determination is helpful because transthyretin amyloid of the bladder has a presentation similar to that of the AL type. The finding of transthyretin amyloid requires no further evaluation. Repeated evaluations for systemic amyloid are unnecessary for patients with the AL subtype in that none
REFERENCES (18)
- et al.
Bilateral localized amyloidosis of the ureter presenting withanuria
J Urol
(1978) - et al.
Amyloidosis of the urethra
J Urol
(1989) - et al.
Unexpected localized amyloidosis: 12 case reports [in French]
Rev Med Interne
(1993) - et al.
Experience with dimethyl sulfoxide treatment for primary localized amyloidosis of the bladder
J Urol
(1986) - et al.
Colchicine therapy in primary amyloidosis of the bladder; a case report
J Urol
(1989) - et al.
Amyloidosis of the urinary bladder
Br J Urol
(1971) Primary amyloidosis of The renal pelvis: a case report and review of literature
Mich Med
(1973)- et al.
Local synthesis of amyloid fibril precursor in AL amyloidosis of the urinary tract
Amyloid
(1998) Ueber locales circumscripted Amyloid in der Harn-blase
Prag Med Wochenschr
(1897)
Cited by (0)
Dr Tirzaman is now with the University of Massachusetts Medical School of Worcester.