DOI: 10.36347/sasjm.2022.v08i03.011

Pages: 167-170

Amyloidosis refers to a group of systemic diseases whose common feature is the extracellular accumulation of insoluble fibrillar proteins in tissues (heart, reins, digestive tract). Several types of amyloidosis have been described; the primary (AL) and the secondary (AA) forms are by far the most frequent. AL amyloidosis is rare but not exceptional disease, with a prevalence of 500new cases per year in France. It is related to be precipitation in tissues of monoclonal light chains of immunoglobulin in the form of fibrils. Its diagnosis is histological based on the biopsy analysis of an affected organ or on non-invasive biopsies (subcutaneous fat, accessory salivary glands, etc) with the detection of amorphous deposits stained by congo red with dichroism and birefringence in polarised light, which is the reference examination. But the diagnostic approach can also be done by thioflavin staining which is very sensitive but not specific for amyloidosis and always requieres a diagnostic confirmation by congo red staining. We report the case of a patient with severe restrictive cardiomyopahy and presenting a set of paraclinical arguments specific for amyloidosis whose diagnosis was made by thioflavin staining.