Systemic Mastocytosis: Symptoms, Risk Factors, Causes, and Treatment
What is Systemic Mastocytosis?
Systemic Mastocytosis is a rare condition that results in too many mast cells building up in your body. A mast cell is a type of white blood cell. These are found in connective tissues throughout your body. They help your immune system function properly and help protect you from disease.
Excess mast cells build up in your bone, skin, digestive tract, marrow, or other body organs when you have this condition. When triggered, these mast cells release substances that can cause signs and symptoms similar to an allergic reaction. This condition can also cause severe inflammation that may result in organ damage. Avoiding its typical triggers, such as spicy foods, alcohol, and certain medications, can help.
Five Main Types of Systemic Mastocytosis
- Indolent. This usually doesn’t include organ dysfunction. Skin symptoms are common, but other organs may be affected, and the disease may worsen slowly over time.
- Smoldering. It is linked with more-significant symptoms and may include organ dysfunction and worsening illness over time.
- Systemic type with another blood or bone marrow disorder. This is considered a severe type that develops rapidly and is often linked with organ dysfunction and damage.
- Aggressive. This is more severe and may show several symptoms that are usually associated with progressive organ dysfunction and damage.
- Mast cell leukemia. This is an extremely rare and aggressive form of this condition.
Signs and Symptoms of Systemic Mastocytosis
The signs and symptoms depend on the part of the body where it occurs. Most of the cells build up in the skin, spleen, bone marrow, and liver skin. Signs and symptoms of systemic Mastocytosis may include:
- Abdominal pain, diarrhea, nausea, or vomiting
- Flushing, itching, or hives
- Bone and muscle pain
- Anemia or bleeding disorders
- Enlarged liver, spleen, or lymph nodes
- Depression, mood changes, or problems concentrating
People have different triggers for this condition, but the most typical causes include the following:
- Skin irritation
- Alcohol
- Exercise
- Insect stings
- Spicy foods
- Certain medications
Talk to your doctor if you have problems with hives or flushing or if you have concerns about the signs or symptoms listed above.
What Causes Systemic Mastocytosis?
Most cases of this condition are caused by a mutation in the KIT gene. Too many mast cells are produced and build up in tissues and body organs, releasing substances such as histamine, leukotrienes, and cytokines that cause inflammation and symptoms. This condition can cause several complications, such as:
- Peptic ulcer disease. Chronic stomach irritation can lead to ulcers and bleeding in your digestive tract.
- Anaphylactic reaction. This severe allergic reaction includes signs and symptoms such as rapid heartbeat, fainting, loss of consciousness, and shock. If you have a severe allergic reaction, you may need an injection of epinephrine.
- Blood disorders. These can include anemia and poor blood clotting.
- Organ failure. A buildup of mast cells in body organs can cause inflammation and damage to the organ.
- Reduced bone density. Because systemic Mastocytosis can affect your bones and bone marrow, you may be at risk of bone problems, such as osteoporosis.
Treatment, Management, and Prevention of Systemic Mastocytosis
The treatment for this condition varies depending on the type of condition, and part of the organ is affected. It focuses on managing the symptoms, regular monitoring, and managing the disease itself. To control the triggers, identify and avoid the common triggers such as foods.
Ranitidine is a prescription used in the treatment and management of this condition. It blocks all the phases of gastric secretion. The recommended dosage of this medicine in adults is 150 mg twice daily or 300mg once daily, given at bedtime. The recommended dose of Ranitidine in pediatric patients is 2 to 4 mg once daily.
Homecare and Lifestyle Support for Systemic Mastocytosis
- Identify and avoid your triggers.
- Treat allergic reactions and always carry your medications to avoid forgetting a dose.
- Follow your doctor’s recommended care and ongoing monitoring.
- Learn as much as you can about your disease and make the best choices.
- Find a team of trusted professionals. Medical centers with specialty teams can offer you information about the disease.