腎臟澱粉樣病變合併腎臟鹿角狀結石-案例分享

徐易廷¹、薛又仁^1,2、黃建榮¹、蕭毅君¹、賴昱維^1,2、邱文祥^1,2

¹臺北市立聯合醫院仁愛院區 外科部 泌尿科；²國立陽明交通大學 醫學院 泌尿學科

None AL Type Renal Amyloidosis Complicated with Staghorn Renal Stone
– A Case Report

Yi-Ting Hsu¹, Thomas Y. Hsueh^1,2, Andy. C. Huang¹, Yi-Chun Hsiao¹, Yu-Wei Lai^1,2,

 Allen W. Chiu^1,2

¹Division of Urology, Department of Surgery, Taipei City Hospital renai Branch, Taipei, Taiwan;

²Department of Urology, School of Medicine, National Yang-Ming Chiao-Tung University, Taipei, Taiwan

Background: Amyloidosis is a rare disease that occurs when amyloid proteins are deposited in tissues and organs. Amyloid proteins clump together and become amyloid deposits. The buildup of these deposits could damage different organs and tissues. Amyloidosis most frequently damages the kidneys, heart, nervous system, liver, and gastrointestinal tract.

Presentation of case: The patient was a 57-year-old female diagnosed of right staghorn renal stone. Computed tomography noted right ureteral pelvic junction thickening. Concerning the possibility of urothelial carcinoma or inflammation reaction, right ureterorenoscopy was performed for biopsy which disclosed pathology of renal amyloidosis eventually. The patient underwent following percutaneous nephrolithotomy and numerous stones were embedded in the pelvis wall with necrosis, calcification and ossification formation. Laboratory data showedβ2-microglobulin: 2.5 mg/L; urine protein/ serum protein electrophoresis: negative, IgA/IgG/IgM: within normal limits, urine light chain: negative. Biochemistry profile suggested that the patient was none immunoglobulin light-chain amyloidosis (AL amyloidosis), which was the most common type. The patient would be further followed up at outpatient department of Hematology.

Conclusions: Depending on the type of amyloidosis, our treatment will focus on slowing the production of amyloid and treating the symptoms of organ damage. Medical therapy including chemotherapy, biological agents, steroid or even autologous stem-cell transplant would be accessible. Most types of renal amyloidosis are not preventable, but managing kidney disease may help delay or avoid further kidney failure.