HEPATIC AMYLOIDOSIS - CASE REPORT AND LITERATURE REVIEW

• Published in: Zdravniški vestnik (Ljubljana, Slovenia : 1992) Slovenia : 1992), 2008-02, Vol.77 (2)
• Main Authors: Knehtl, Masa, Ceranic, Davorin, Urlep, Dejan, Glumbic, Ivana, Kavalar, Rajko, Skalicky, Marjan, Skok, Pavel
• Format: Article
• Language: Slovenian
• ISSN: 1318-0347; 1581-0024

BACKGROUND Amyloidosis is a disorder of protein metabolism that leads to extracellular deposition ofinsoluble proteinaceous material. Clinically important are amyloidosis AL and AA. Primaryor AL form is associated with immunocyte dyscrasia, multiple myeloma, macroglobulinemiaor monoclonal gammopathy, the precursor protein are immunoglobulin lightchains. Amyloidosis AA or reactive amyloidosis is associated with chronic inflammatorydisease.Patients and The authors are presenting a 52-year old man who was transferred to their department formethods diagnostic proceedings of anaemia and liver disease from the department of orthopaedics,where he was treated for compression fractures of the spine. Unfortunately, during the firsthospitalisation the patient refused liver biopsy. The patient died due to spleenic ruptureand exsanguination into abdominal cavity. At autopsy, amyloidosis was found withamyloid deposits in the liver and other organs. In the article, different forms of this diseasewith grim prognosis are described. CONCLUSIONS The authors presented a patient with primary amyloidosis which led to fatal complication.According to the literature, patients with this form of amyloidosis have median survival of1-2 years from establishing the diagnosis