Mast Cell Activation Syndrome - Causes, Symptoms, and Treatment

Introduction:

Mast cells are a type of blood cell that is a part of the immune system. Apart from helping fight off infections, they are also involved in allergic reactions. The lifespan of mast cells is longer than normal cells, and they grow in the bone marrow, gastrointestinal tract, skin, and airways.

Mast cells release certain chemicals called mediators on coming in contact with an allergen or substances that cause allergies. Some of these chemicals are released immediately, and some take longer. One such chemical is histamine which might induce allergy symptoms. These symptoms include-

• Itchy skin.

• Swollen skin.

• Mucus buildup.

• Tightened airways.

• Wheezing.

• Headaches.

• Expanding blood vessels.

• Anaphylaxis (life-threatening allergic reaction).

In healthy individuals, these chemicals have healing and protecting functions. But for someone with mast cell activation syndrome, they have negative effects.

Mast cells can sometimes get activated or triggered by certain things like-

• Insect venom.

• Infections.

• Medications.

• Reptile venom.

• Exercise.

• Fragrances.

• Stress.

• Food.

People with mast cell activation syndrome exhibit lots of allergy symptoms and might have lots of episodes of anaphylaxis (serious, potentially fatal allergic reaction) without any clear cause.

Sometimes mast cell activation syndrome can be confused with mastocytosis. They both are types of mast cell disease but two different conditions. Mastocytosis is a rare genetic condition that causes the mast cells to produce an abnormal amount and gather in certain organs such as the liver, spleen, gut, and skin. This causes too many mast cells and random chemical releases that can be overwhelming for the body, making one sick. This rare disease can be responsible for some skin growths and a blood disorder called leukemia.

What Causes Mast Cell Activation Syndrome?

The exact reason that causes mast cell activation syndrome is unknown. Therefore, it is sometimes referred to as an idiopathic condition because it is not related to any clear allergy or cause and no other disease causes it. Individuals suffering from this syndrome might have a hard time pinpointing the exact thing that is triggering the allergic reactions in their bodies. There might be many new triggers coming up.

What Are the Signs and Symptoms of Mast Cell Activation Syndrome?

Many different symptoms might be affecting a lot of the body systems, sometimes all at once. These can occur after eating some foods, smelling certain fragrances, exercising, and many other things. The primarily affected areas in your body include the skin, nervous system, gastrointestinal tract, and heart. The number of mediators that get released will determine the severity of the symptoms, which may be mild to life-threatening.

Some key signs include the following -

• Symptoms show up in more than one system.

• Symptoms that appear are cyclical.

• Many different triggers can be present.

• Difficulty in identifying the triggers.

• Dramatic changes in symptoms.

The symptoms include -

• Skin - Sweating, swelling, itching, hives, rash, and flushing (skin turning red).

• Eyes - Itching, watering, and irritated.

• Nose - Itchy and running nose.

• Mouth and Throat - Itching, swelling in the tongue or lips, and swelling in the throat.

• Lungs - Wheezing and trouble breathing.

• Heart - Low blood pressure and fast heart rate.

• Abdomen - Cramps, pain, nausea, vomiting, diarrhea, and constipation.

• Nervous System - Headache, confusion, fatigue, and fainting.

In severe cases, one may experience anaphylaxis or anaphylactic shock (severe and life-threatening allergic reaction), an emergency situation requiring immediate attention. The symptoms include-

• Lightheadedness.

• Weak pulse.

• A rapid drop in blood pressure.

• Trouble breathing or quick and shallow breathing.

• Confusion.

• Loss of consciousness.

How to Diagnose Mast Cell Activation Syndrome?

The diagnosis of mast cell activation syndrome is difficult because it has many symptoms that may mimic other conditions, and it is difficult to know the exact cause.

There are three specific things that the healthcare provider will look for-

• Allergy symptoms or other symptoms affecting more than two organ systems of the body that are recurring or chronic in nature.

• Higher than normal levels of mast cell mediators in the blood. These mediators are- histamines, tryptase, leukotriene, or prostaglandin.

• Getting better after taking certain medications, such as Antihistamines, that block the release of these mediators.

If all three criteria mentioned above are met, then it is likely that the doctor may diagnose mast cell activation syndrome.

How to Treat Mast Cell Activation Syndrome?

Currently, there is no cure for mast cell activation syndrome. However, the symptoms can be managed.

Treatment can include-

• H1 or H2 Antihistamines- These drugs are useful in blocking the effects of histamine, which is the primary mediator released by the mast cells.

• Histamine Type 1 Receptor Blockers- Cetrizine and Loratadine. They help with itching and stomach pain.

• Histamine Type 2 Receptor Blockers- Ranitidine and Famotidine. They help with abdominal pain and nausea.

• Aspirin- Can reduce flushing.

• Mast Cell Stabilizers- Omalizumab. This may help in preventing the release of mediators and thereby lowering the episodes of anaphylaxis.

• Antileukotrienes- Montelukast and Zafirlukast. These help with wheezing and stomach cramps.

• Corticosteroids- These should be considered as a last resort for treating edema, wheezing, or hives.

If one develops an anaphylactic reaction, they will need an epinephrine injection or the healthcare provider might give an auto-injector Epinephrine pen (EpiPen) to use in case of emergencies.

Stress can also result in the activation of the mast cells. Therefore it is important to manage your stress levels.

Following is a list of certain foods that are high in histamine and better to be avoided-

• Hard cheese.

• Spinach.

• Sausages.

• Fish.

• Alcohol.

Conclusion:

Mast cell activation syndrome is a condition that can have several unexpected allergy-like symptoms that interfere with your daily life. It can also cause anaphylaxis which is a serious life-threatening allergic reaction and requires immediate attention. While the exact cause of mast cell activation syndrome is unknown, proper diagnosis and treatment can be helpful in managing the condition.