Primary Amyloidosis and a Case Report

Background: Primary amyloidosis is the most common form of systemic Amyloidosis. Amyloidosis is the results of fibril formation due to plasma cell dyscrasia or rarely monoclonal gammopathy of uncertain significance (MGUS). Clinical manifestations are proteinuria, cardiomyopathy, peripheral neuropathy, large tongue and shoulder fat pad enlargement and hepatosplenomegaly. Methods: The presented patient is a 42-year old woman with clinical features of a dull epigastric and left upper abdominal pain from one year ago. Her family history and past medical history was normal except for an exertional dyspnea. In the physical examination, paleness, large tongue with indentations around it and hepatosplenomegaly were obvious, during admission, she had rectorrhagia, polipectomy led to severe hemorrhage. Echocardiography performed and revealed the typical sign of sparkling pattern with restrictive cardiomyopathy. Findings: In bone marrow evaluation plasma cells were 8%. Serum protein electrophoresis showed that monoclonal gammopathy near 2 grams in weights and all data confirm monoclonal gammopathy of uncertain significance. Duodenal biopsy verified an amyloid deposition which was confirmed by Congo red coloration. There were no data to support secondary amyloidosis in our wide investigation and patient was treated with the diagnosis of primary amyloidosis due to monoclonal gammopathy of uncertain significance. Conclusion: Primary amyloidosis secondary to monoclonal gammopathy of uncertain significance is a rare disease which should be mentioned in chronic multiorgan disorders. Key words:: Primary amyloidosis, Monoclonal gammopathies of uncertain significance, Hepatomegaly.