Adrenal gland Amyloidosis : Histopathology

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Adrenal gland Amyloidosis : Shotgun Histology
What is Adrenal Amyloidosis?
An amyloidosis is a group of disorders in which the ‘amyloid protein’ builds up in many organs and tissues of the body. The term “amyloid” is used for protein molecules of any type that stick together owing to misfolding (or incorrect formation of the proteins)
These protein aggregates are abnormally-formed and arranged into fibrils (slender fibers). An amyloid build-up can occur locally in only one organ or may occur throughout the body. When the amyloid proteins get deposited in the adrenal glands, the resulting condition is known as Adrenal Amyloidosis
The adrenal gland is a small gland that is located one on top of each kidney
These glands secrete hormones such as male sex hormones, aldosterone, and cortisol
The sex hormones secreted by the glands play a role in puberty; aldosterone regulates salt and water in the body; and, the cortisols regulate metabolism
There are 5 major types of Amyloidosis, all of which can lead to Adrenal Gland Amyloidosis:
AL amyloidosis that occurs when the bone marrow produces too much amyloid protein, creating light (L) chains
AA amyloidosis, when amyloid proteins build up secondary to a chronic disease
Hereditary amyloidosis: It is an inherited form of the disease; the amyloid build-up primarily affects the kidneys and nerves
Wild-type ATTR amyloidosis: This form leads to amyloid protein deposits in elderly adults, particularly in the heart and tendons
Dialysis-related amyloidosis: This occurs in individuals who have undergone dialysis for a long time. The amyloid protein tends to get deposited in the tendons and joints
Adrenal Amyloidosis is commonly associated with systemic AA amyloidosis. It may also occur in AL- and hereditary- forms of amyloidosis
If Adrenal Amyloidosis occurs in systemic AA amyloidosis, it can occur at any age. When associated with AL or hereditary subtypes, older adults are commonly affected
Generally, the manifestation of symptoms of Adrenal Amyloidosis does not occur, unless extensive amyloid deposition has occurred in both the glands. When symptoms do occur, the affected individuals may present with decreased appetite, joint and neck pain, decreased sex drive, and low blood pressure
The treatment involves effective management of amyloidosis and the underlying condition causing it. If systemic amyloidosis causing Adrenal Amyloidosis involves major organs, the prognosis is guarded
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