Akademik Veri Yönetim Sistemi
EUROPEAN GERIATRIC MEDICINE, cilt.5, ss.247, 2014 (SCI-Expanded, Scopus)
A case of primary amyloidosis presenting with massive proteinuria and localized gastrointestinal involvement M. Cengiz1, S. Yavuzer1, H. Yavuzer2, M. Yuruyen3, F. Demirdag2, Z. Kara2, A. Doventas2, Y. Karter1, A. Yaldiran1, T. Beger3, D.S. Erdincler2 1Department of Internal Medicine, Cerrahpasa Faculty of Medicine, Istanbul Univer, Istanbul, Turkey; 2Department of Geriatrics, Cerrahpasa Faculty of Medicine, Istanbul University, Istanbul, Turkey; 3Cerrahpasa Medical School, Istanbul University, Istanbul, Turkey Amyloidosis involving different parts of gastrointestinal system were reported in the literature and there has been only one case of ampullary involvement so far. We report on a case of a female patient diagnosed at advanced age presenting with massive proteinuria and bile duct dilatation. Case presentation: A 82-year-old female presented with a 7-month history of edema in the lower extremities. Physical examination; minimal ascites, palpable liver 2cm below the left costal margin and Murphy positivity were detected. The patient’s biochemical parameters were as follows: hematocrit 32.7%, hemoglobin 10.5mg/dL, creatinine 1.6mg/dL, albumin 1.5g/dL, 24h total urinary protein 14g. IgG/lambda monoclonal band (Lambda 3510mg/L, 9002100) was detected in immunofixation electrophoresis. Primary AL amyloidosis was diagnosed by percutaneous renal biopsy. Mild dilatation of common bile duct and Wirsung canal and bulging of papilla were demonstrated by endoscopic USG and ERCP (Figure 1). Biopsies showed that amyloid deposition. Melphalan was not be able to given to the patient due to poor general condition, thus only 2 cycles of chemotherapy including dexamethasone (20mg/m2) were administered. Because of the progression of the condition and intervening infections it was unable to continue the treatment. The patient died six months after the initial diagnosis. Conclusion: Our case becomes notable as being the case of a primary amyloidosis accompanied by nephrotic syndrome with ampullary involvement, which caused rapid progression of widespread ascites, hydropic gallbladder, bile duct dilatation, although the diagnosis is established shortly after admission. It also comes into prominence as being the only case reported from Turkey