Wat betekent al bij Amyloidose?
AL amyloïdose is een eiwit stapelingsziekte die ontstaat door een voorstadium van beenmergkanker. De kankercellen zijn plasmacellen die een zeer afwijkende afweerstof aanmaken. Deze afweerstof is de oorzaak van de eiwitstapeling.
Wat is cardiale Amyloidose?
Amyloïdose is een aandoening waarbij bepaalde eiwitten (uit verschillende bronnen) verkeerd gevouwen worden en in het lichaam niet goed afgebroken worden. Dit leidt ertoe dat eiwitten zich ophopen in verschillende organen zoals het hart.
What is amyloidosis?
Overview. Amyloidosis (am-uh-loi-DO-sis) is a rare disease that occurs when a substance called amyloid builds up in your organs. Amyloid is an abnormal protein that is produced in your bone marrow and can be deposited in any tissue or organ. Amyloidosis can affect different organs in different people, and there are different types of amyloid.
How is the diagnosis of amyloidosis (hives) made?
Diagnosis of amyloidosis generally requires tissue biopsy. The biopsy is assessed for evidence of characteristic amyloid deposits. The tissue is treated with various stains. The most useful stain in the diagnosis of amyloid is Congo red, which, combined with polarized light, makes the amyloid proteins appear apple-green on microscopy.
Is amyloidosis a protein misfolding disease?
Amyloidoses can be considered protein misfolding diseases. The vast majority of proteins that have been found to form amyloid deposits are secreted proteins, so the misfolding and formation of amyloid occurs outside cells, in the extracellular space.
What is the pathophysiology of hereditary amyloidosis?
Hereditary amyloidosis is characterized by the deposit of an abnormal protein called amyloid in multiple organs of the body where it should not be, which causes disruption of organ tissue structure and function. In hereditary amyloidosis, amyloid deposits most often occur in tissues of the heart, kidneys, and nervous system.