Laryngeal Amyloidosis - Symptoms and Treatment

Introduction:

The amyloid deposition looks like a yellowish-waxy substance with no distinct border. Most of the time, surgical management of laryngeal amyloidosis is not successful. Patients suffering from this condition may experience breathy or impaired voice. The folds created due to amyloid deposition cause a change in the voice.

What Is Laryngeal Amyloidosis?

The deposition of protein in any tissue or organ changes its architecture. This causes abnormal cell function. The human body produces proteins, some of which make up genetic construction like DNA. Each protein has a specific shape and interacts with other cells. The cell functions disrupt when more misfolded proteins are made and get accumulated in the tissues. Amyloid is unbreakable and difficult to break down. They are incurable and can be treated for underlining conditions. The type of protein accumulated determines the condition of the disease. For example, immunoglobulin light-chain (AL) amyloidosis is the commonest form of amyloidosis present.

What Are the Different Types of Amyloidosis Present in Organs?

Various classification of amyloidosis has been made depending on the primary or secondary lesion or otherwise based on acquired and hereditary factors. Such as

1. Systemic Amyloidosis: These are formed anywhere in the body. Plasma cell clones cause them. It affects all the organs except the brain.

2. Localized Amyloidosis: These are formed in a localized area. The lesion is acquired through a plasma cell clone. It affects the tracheobronchial tree, lungs, urinary bladder, skin, and other organs. Treatment is done by localized therapy.

3. ATTRwt (Wild Type Amyloid Transthyretin): These are caused by wild-type transthyretin. It can be either acquired or developed during the aging process. It affects the heart, soft tissues, and lungs. It leads to heart failure, and hence treatment for heart failure is given.

4. ATTRcm (Transthyretin Amyloid Cardiomyopathy): These are caused by mutated transthyretin. This could be due to hereditary factors. It involves the heart, peripheral nervous system, and anterior nervous system. It damages the involved organs, like the heart, and causes peripheral neuropathy and liver damage, causing liver transplantation surgery.

5. AA (Secondary Amyloidosis): This is caused by apolipoprotein serum amyloid. It could be acquired or caused by a response mechanism seen in chronic inflammation. It affects the kidneys, heart, liver, and lungs. It causes nephrotic syndrome. Treatment is undertaken to treat the underlining conditions.

Which Is the Most Commonest Form of Amyloidosis Seen Among Patients?

The commonest form of amyloid deposition seen in patients is the localized form. The factors resulting in such conditions are:

• Amyloid fibrils are lightly chained in local lesions.

• It rarely spreads through the circulatory system.

• It appears in the regions of nails, larynx, lung, bowel, orbit, and urinary tract.

• Recurrent rates are high if any residual tumor is left.

• It is usually benign.

• If left untreated, it can damage the organs.

• It can be treated locally by laser surgery or surgical excision.

What Are the Symptoms Associated With Amyloidosis?

Symptoms depend on the site of the lesion and its progression. Some of the symptoms seen are

• Dysphonia (discordant sound).

• Hoarseness of sound.

• Persistent cough.

• Associated vasculitis is seen at the site of the lesion.

• Dyspnea or shortness of breath.

• Thickening of the walls of the epiglottis.

• Aryepiglottic folds are seen.

• Nodular ventricular bands are seen.

• Nasal obstruction.

• A wheezing sound is made from the larynx or the presence of stridor, especially while breathing.

• Mucosal ulcers.

• Laryngitis.

• Infection of the otitis or ear.

• Hemoptysis or cough with bloody sputum.

What Are the Risk Factors Associated With Amyloidosis?

Amyloid deposition is seen happening all over the body. The most common site of occurrence is the neck airway spaces. The condition or population which can be risky for amyloidosis are:

• Smoking can trigger the condition.

• Older people between the ages of 50 and 70 years are more likely to develop this condition.

• It is more commonly seen in the male population.

• Alcohol consumption is a predisposing factor.

• The occurrence of plasma cell reaction causes the release of inflammatory antigens.

How To Diagnose Laryngeal Amyloidosis?

Various investigation procedures are carried out to diagnose the condition, such as

• Computed Tomography Scan (CT): This is done to evaluate the soft tissue lesions.

• Microlaryngoscopy: It is performed to check for the extent of the tumor and remove any unwanted growth from the mucosal folds, especially the larynx area.

• Histopathological Studies: This is done by obtaining biopsy tissue and helps in confirming amyloidosis.

• Chest X-ray: The presence of any growth is checked.

• Echocardiogram: Damage to the heart cells is evaluated.

• Blood Tests: Various antigens released in the blood are checked.

• Liver Function Test: The damage to liver cells is evaluated.

• Urine Analysis: The level of protein and creatinine excreted through urine is evaluated.

• Kidney Function Test: The normal function of the kidneys is evaluated.

What Are the Treatment for Laryngeal Amyloidosis?

Treatment modality depends from person to person and the extent of the lesion. Since it is more commonly found in the older generation, other underlying disease conditions are evaluated. Any treatment started will be based on controlled body parameters. Here are some available treatments:

• Tracheostomy: Airway management is done locally, excising the trachea, and a tracheostomy tube is inserted for breathing.

• Laryngectomy: The entire larynx is removed along with the sound box- the larynx, and an airway are created for breathing.

• Surgical Management: It is done by endoscopic carbon dioxide laser excision.

• Corticosteroid Therapy: Corticosteroid drugs are administered, which can suppress the immune system.

• Radiotherapy: High doses of radiation are induced at the site of cancer cells or abnormal growth mass to kill it.

• Treating Underlying Conditions: Treating the underlining conditions like heart failure and kidney failure.

Conclusion:

Thousands of proteins are present in the body that helps in cellular function. Amyloidosis leads to protein aggregation and deposition in various tissues altering their function. More than thirty different proteins have been known to misfold and cause amyloidosis. This can be due to genetic, acquired, or aging factors.