Unexpected systemic amyloidosis in patient with restrictive cardiomyopathy Case report

Gabrijela Stanic



Systemic amyloidosis is a rare disease and it is caused by deposition of insoluble abnormal fibrillary proteins known as amyloid in extracellular space. Protein deposits are components of immunoglobulins produced by plasma cells and B–lymphocytes in response to antigenic stimulation. Three different  forms of amyloidosis can be distinguished, the primary (AL), secondary (AA) and family form. Localized amyloidosis occurs in the pancreas in type 2 diabetic patients and in patients on chronic hemodialysis. The clinical presentation in amyloidosis depends on the affected organs. Symptoms are non-specific and included fatugue, weight loss, nephrotic syndrome, restrictive cardiomyopathy with thickening of the interventricular septum and ventricular wall, peripheral neuropathy, hepatomegaly, macroglossia, purpura and bleeding diathesis. In these article we presented the unexpected systemic amyloidosis in a patient with restrictive cardiomyopathy and without malignant and hematological diseases who suffered from dyspnea, weakness, fatigue, loss of appetite and cough.

Diagnosis of systemic amyloidosis was made upon histological samples after autopsy.


Key words: amyloid, systemic amyloidosis, gastrointestinal tract, cardiomyopathy, kidney failure

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