Amyloidosis is characterized by the abnormal accumulation of amyloid in the extracellular spaces of tissues and organs. Normally antibodies, are produced by cells in the bone marrow and destroyed after their function is served. Amyloidosis occurs when amyloid proteins are not recycled properly and accumulate in tissues and organs, resulting in severe structural and functional organ damage.
Amyloidosis is classified into three major forms: primary (AL) amyloidosis, secondary (AA) amyloidosis, and hereditary (ATTR) amyloidosis. AL amyloidosis is the most common and occurs due to the abnormal production of light chains by plasma cell neoplasms (PCN). The PCN cells secrete abnormal antibodies into the blood, which eventually accumulate in tissues, resulting in the symptoms of amyloidosis. AA amyloidosis occurs due to complications from a chronic inflammatory or tissue destructive disease. ATTR amyloidosis occurs due to mutations, and is the only type that is inherited. The most common form of ATTR is senile or wild type amyloid (ATTRwt).
The severity of amyloidosis largely depends on which organs are affected and how much amyloid has accumulated. Amyloidosis can affect different organs including the heart, kidneys, liver, spleen, nervous system, and gastrointestinal tract. The most life-threatening cases occur in patients with amyloid deposits in the heart.
Although amyloidosis is considered a rare disease, it is underdiagnosed and patients often visit many doctors before receiving an appropriate diagnosis. Treatments are available to help manage patient symptoms, limit the production of further amyloid proteins, and support the affected organs. Untreated, there is a poor prognosis for amyloidosis. Therefore, doctors at NYP place a high priority on diagnosing and treating patients in the early stage of the disease before organs are seriously affected.