A guide to systemic mastocytosis and liver complications

Photo shows doctor sitting behind human liver model on his desk/Getty Images
Doctor sits behind human liver model on his desk/Getty Images
People living with systemic mastocytosis (SM) can experience liver abnormalities due to mast cells infiltrating bone and internal organs.

People living with systemic mastocytosis (SM) can experience liver abnormalities due to mast cells infiltrating bone and internal organs, including the liver, spleen and lymph nodes.

When mast cells accumulate in the organs, it can lead to inflammation, tissue damage or organ failure. Minor liver complications can often be managed through lifestyle and diet changes and medication. If liver failure occurs, a transplant is the only cure.

What is the role of mast cells in SM?

SM is a chronic white blood cell disease that affects the mast cells, part of the body’s immune system. Mast cells are made in the bone marrow and release chemicals such as histamine, cytokines and growth factors into the bloodstream when they detect bacteria or allergens in the body.

These immune responses can trigger symptoms such as flushing of the face, skin itchiness, headaches, abdominal cramps, heart palpitations, joint pain, low blood pressure and potentially, anaphylaxis. In SM, mast cells proliferate uncontrollably and release excessive amounts of histamine.

There is no cure for SM, and treatment focuses on avoiding symptom flare-ups through triggers such as changes in temperature, physical or emotional exertion, alcohol, certain foods and medication, insect bites or stress.

Learn more about SM treatment and care

Identifying liver complications

Complications of the liver associated with SM include swelling of the liver or hepatomegaly, portal hypertension and ascites, sclerosing cholangitis or Budd-Chiari syndrome.

In patients with unexplained liver problems, swelling of the liver or hepatomegaly may lead to an SM diagnosis. Signs of an enlarged liver include abdominal pain, fatigue, nausea, vomiting and jaundice.

Diagnosis of hepatomegaly involves several steps, starting with a physical exam, then a blood test to check liver enzymes, a CT, ultrasound or MRI and potentially a liver biopsy.

Organ damage, dysfunction and even failure more often occur in advanced SM, or in the more severe variants of the disease, such as aggressive SM.

Managing liver complications with systemic mastocytosis

Liver damage can be partially slowed through diet and lifestyle changes, and by effectively avoiding triggers to reduce the symptom burden of SM.

Medications such as corticosteroids and immunosuppressants are used in the treatment and management of liver complications. Of course, each patient’s treatment plan may look different, depending on their symptoms and the advice from their doctor.