Protein Losing Enteropathy (PLE)
What Is Protein Losing Enteropathy (PLE)?
Protein losing enteropathy (PLE) is when too much lymph leaks into the intestines, which are in your gastrointestinal (GI) tract. Lymph is a clear fluid in your body that carries fats and proteins that help fight infections. PLE is often caused by problems with the lymphatic system.
PLE is a condition in which there is abnormal intestinal absorption of nutrients and inappropriate leakage of important proteins from the gastrointestinal (GI) tract.
Many cases of PLE are related to disorders of lymphatic flow.
What Causes PLE?
Lymphatic flow problems in the GI tract can lead to lymphatic channels in the intestines that are too big. This can happen because of lymphangiectasia, meaning part of the GI tract is not shaped or does not work like it should. It may also be caused by right heart failure.What Are Some Symptoms of PLE?
Symptoms of PLE include:
- Swelling in the arms or legs (limbs)
- Diarrhea
- Belly (abdominal) pain
- Bloating
- Fluid buildup in the abdomen (ascites)
- Not wanting to eat or is not able to eat
- Blood does not clot like it should
- Low blood albumin level
- Low lymphocyte levels (a type of white blood cell)
- Increased risk of infection
How Is PLE Diagnosed?
Experts at Nationwide Children’s Hospital can take direct images of the lymphatic system using advanced imaging technologies, like fluoroscopy (moving X-ray) and magnetic resonance imaging (MRI). This helps providers find exactly where a lymphatic leak is happening. Knowing this will lead to a specific treatment for each patient. Imaging is usually done while a patient is asleep under sedation. An MRI contrast dye is put into (injected) parts of the lymphatic system like the groin, liver or around the intestines. Each of these areas show a different part of the lymphatic system to get the best picture possible when imaging. If a certain leak is found, patients may be able to have their leaking lymphatic channel(s) closed. This is called selective embolization.How Is PLE Treated?
There are a lot of pieces to the treatment puzzle. Treatment may include:
- Medicines like steroids and diuretics to lessen the swelling in the intestines
- Diet changes
- Treating low red blood cell count (anemia)
- Replacing a protein made in the liver (albumin)
- Other targeted therapies
The Lymphatic Disorders Program at Nationwide Children’s works with other teams throughout the hospital to create a personal treatment plan to help treat PLE, which can be hard to manage.
