Primary Localized Amyloidosis of the Bladder: A Case of AL (λ) Amyloid Protein and Combination Therapy Using Dimethyl Sulfoxide and Cepharanthin

Abstract

We report a case of primary localized amyloidosis of the bladder with amyloid deposits which was characterized as being of immunoglobulin light chain origin (AL) including Λ type (A Λ) and P component (AP) using the KMnO₄ pre-treatment method and immunohistochemical procedures. The patient was treated successfully with intravesical dimethyl sulfoxide instillation and oral administration of high-dose cepharanthin after transurethral resection. Combination therapy with dimethyl sulfoxide and cepharanthin was shown to be useful for primary localized amyloidosis of the bladder.

Anurea caused by primary amyloidosis of the lower third of the ureters, the ureterovesical junction and the urinary bladder

“…An interesting combination therapy with intravesical instillation of DMSO and oral administration of high-dose cepharanthin was demonstrated to be efficient for the treatment of primary localized amyloidosis of the urinary bladder [17]. However, the effectiveness of these non-operative strategies has been questioned in the past [18,19], especially when cases with diffuse primary amyloidosis are concerned.…”

Localized Amyloidosis of the Upper Urinary Tract: A Case Series of Three Patients Managed with Reconstructive Surgery or Surveillance

“…2,3 Medical management with intravesical dimethyl sulfoxide, oral colchicine, and cepharanthine has been reported in rare cases with limited success. [16][17][18] Nephroureterectomy is the predominant treatment for upper urinary tract amyloidosis, but local resection with reimplantation has been reported. 7,19 There are no reported cases of any successful medical interventions in the treatment of upper tract amyloidosis.…”

Constant Region of a κ III Immunoglobulin Light Chain as a Major AL‐Amyloid Protein

“…A very faint labelling of a band corresponding to about 14 kDa can also be seen. Antiserum to BJ-VAG [5][6][7][8][9][10][11][12][13][14] labels the upper two bands of amyloid fibrils VAG strongly, while the bands at 14 kDa and lower are close to negative (Fig. 2, lane 6).…”

“…Immunoglobulin light chains can also give rise to an uncommon form of localized, often tumour-like, amyloid form described for many different tissues including skin [6], breast [7], brain [8], respiratory tract [9,10] and urinary bladder [11]. In this form of AL-amyloidosis, the monoclonal protein is most likely produced at the site of deposition by a clone of plasma cells, present locally in the tissue [10].…”