Protein Losing Enteropathy (PLE)

What Is Protein Losing Enteropathy (PLE)?

Protein-losing enteropathy (PLE) occurs when albumin and other protein-rich materials leak into your intestine. Albumin is the most abundant protein in your blood. It has many functions, including transporting hormones and retaining water in your bloodstream.

Symptoms of PLE

If you have PLE, you will have hypoalbuminemia (low level of the albumin in blood) and may experience the following symptoms:

• Diarrhea
• Tissue swelling (edema)
• Ascites (excess fluid trapped in your abdomen)
• Pleural and pericardial effusions (excess fluid around your heart)
• Hypoproteinemia (lower than normal protein levels in your body)
• Severe malnutrition

Hypoalbuminemia is also associated with poor surgical outcomes and an increased risk of death.

Causes of PLE

PLE has been linked to more than 60 diseases and disorders, including congenital heart disease, cancers, immune disorders, inflammation of the gastrointestinal tract and all intestinal and digestive diseases. Intestinal lymphangiectasia (dilated intestinal lymphatics) is often found in patients with PLE and can be explained by a condition that is called lymphatic congestion.

One of the ways that congestive heart failure (CHF) results in PLE only recently came to light. One of the hallmarks of CHF is an elevated central venous pressure. It is well known, that the increased central venous pressure causes liver congestion, further resulting in increased production of liver lymph, which is very rich with albumin.

Using a technique called liver lymphangiography, our clinical research team discovered that there is a leakage of albumin-rich liver lymph into the intestine through the abnormal communications between liver/retroperitoneal lymphatics and duodenum. They then realized that embolization, a technique that uses medical glue to block lymphatic vessels, could be an effective treatment for PLE. The researchers studied the effectiveness of therapeutic imaging and embolization of the affected vessels. The study determined that embolization of the lymphatic connections between liver and intestine results in sustained improvement of PLE symptoms and albumin levels in patients with imaging and endoscopic confirmation of lymphatic leakage into the duodenum.

Diagnosis of Protein Losing Enteropathy (PLE)

If you are suspected to have PLE, your doctor can confirm your diagnosis by measuring albumin and protein levels in your blood. PLE is then confirmed by the presence of alpha-1-antitrypsin, a sensitive protein marker, in your feces.

In appropriate patients, dynamic contrast-enhanced MR lymphangiography can be performed to demonstrate the status of the central lymphatic system where the liver lymph drains.

 

Treatment of PLE at Penn

If you have PLE, your symptoms may be resolved by embolization of the abnormal communications between your liver lymphatics and intestine.

The embolization is performed by injecting surgical glue diluted with x-ray visible contrast agents through a very small needle that's inserted in your liver lymphatic vessels. Fluoroscopic and ultrasound guidance is used to direct the needle. The glue then propagates through the retroperitoneal lymphatic ducts until it plugs the holes in the duodenum.

 

Fluoroscopy image of the duodenum after lymphatic embolization, demonstrating filling of the dilated lymphatic channels in duodenal wall.

In addition to the x-ray dye, your doctor may also inject a special blue dye into your liver lymphatic while performing an endoscopy. This may help to further confirm the location of intestinal leakage.

Penn Programs & Services for Protein Losing Enteropathy (PLE)