What is Peliosis Hepatis?
Peliosis hepatis is a blood vessel related disease where parts of the liver abnormally grow and multiply. This results in the blood vessels of the liver becoming overstuffed and forming spaces or cavities in the liver. This disease often goes unnoticed and is usually discovered by chance during abdominal tests or autopsies.
What Causes Peliosis Hepatis?
Peliosis hepatis is a medical condition that was first identified by E. Wagner in the late 18th century in patients with tuberculosis infections. Today, it is known to be linked to a range of different situations, including certain medications, health conditions, surgeries, cancers, and autonomous (or ‘self-targeting’) conditions.
Medications that are toxic treatments, such as immunosuppressants like 6-mercaptopurine, azathioprine, methotrexine, and tamoxifen, have been connected to this condition. In terms of infections, tuberculosis, HIV, and bacillus are all associated with peliosis hepatis.
The occurrence of this disease in individuals who already have related conditions varies, ranging from 0.2% to 22%. Even though it has been very rarely documented, individuals with a known inherited muscular disorder called X-linked myotubular myopathy seem to be more likely to develop peliosis hepatis. This implies that there could be a genetic factor involved.
Risk Factors and Frequency for Peliosis Hepatis
Peliosis hepatis is a condition that is being reported more frequently due to advancements in medical imaging and increased number of autopsies. Most of the time, the cause of peliosis hepatis is unknown. However, it’s commonly found in people who have recently had a kidney transplant. Regardless of gender, anyone can develop this condition. While it typically affects adults, it is also diagnosed in children.
Signs and Symptoms of Peliosis Hepatis
Peliosis hepatis is a medical condition that often goes unnoticed because many patients do not experience symptoms. Sometimes, it’s discovered when patients undergo liver function tests, and abnormal results hint towards its possibility. However, diagnosis can be hard because some patients may have perfectly normal liver function test results.
Although less common, symptoms related to liver or gallbladder problems may arise, such as abdominal pain (especially on the right upper side), yellowing of the skin (jaundice), and signs of increased blood pressure in the portal vein, a large vein that carries blood from the digestive organs to the liver.
The symptoms of peliosis hepatis do not seem to correlate with the severity of the disease. Therefore, patients may show different symptoms depending on the specific causes and progression of the disease. Because of this, and because their general laboratory findings are usually unremarkable, patients may not seem critically ill. This can sometimes lead to overlooked diagnoses.
In rare cases, severe abdominal pain can be a sign of internal bleeding in the abdomen. Patients may also show signs of infection like fever and abdominal pain, which calls for immediate imaging to rule out an infection in the bile-containing portion of the abdomen.
If patients exhibit the above symptoms and also have weight loss, swollen lymph nodes, and diarrhea, a specific form of the disease caused by Bartonella bacteria (often transmitted through household pets) should be considered.
Testing for Peliosis Hepatis
If a doctor suspects that a patient has a condition called peliosis hepatis, the first step they usually take is to conduct lab tests, specifically a liver panel test. This test helps understand how well the liver is functioning but may or may not show any changes even if the patient has peliosis hepatis.
After doing these laboratory tests, the doctor usually moves on to imaging studies. The most common technique used to capture pictures of the liver is ultrasonography, a tool that uses sound waves to create images of internal organs.
In some cases, the doctor may need to do additional imaging tests such as a computed tomography (CT) scan, magnetic resonance imaging (MRI) scan of the abdomen, or angiography which is an x-ray to visualize blood vessels. However, these tests aren’t always necessary. If the ultrasound shows suspicious findings that point towards peliosis hepatis, usually, what follows next is a biopsy.
A biopsy is when a small piece of the liver is removed and examined under a microscope to confirm the diagnosis of peliosis hepatis. This procedure is deemed the most effective in identifying this condition with certainty.
Treatment Options for Peliosis Hepatis
Peliosis hepatis is a condition that is often discovered accidentally or during an autopsy, as it does not usually present with noticeable symptoms. Because of this, it often isn’t necessary to take any specific actions to treat it. However, depending on its cause, there might be some treatments that could help.
For example, if the condition is likely caused by medications you are currently taking, discontinuing that medication could help. Similarly, if the condition is thought to stem from a suppressed immune system due to certain kinds of therapy, switching to a different type of immunosuppressing therapy might be beneficial.
In cases where other treatable conditions might be contributing to the development of peliosis hepatis, addressing those conditions could also lead to regression of the peliosis hepatis. For instance, if the condition is caused by specific bacteria, antibiotics have been shown to help.
Currently, there isn’t a general agreement among medical professionals about whether routine scans to monitor the progression of asymptomatic (not causing any symptoms) peliosis hepatis are needed. However, some experts suggest that an ultrasound check-up once a year or even more often could be adequate.
What else can Peliosis Hepatis be?
Peliosis hepatis, a condition affecting the liver, is hard to diagnose because its appearance can be similar to other medical conditions when viewed through microscopic and radiographic studies. When observed under a microscope, this condition may resemble other conditions including portal vein thrombosis, heart failure, and nephrotic syndrome, which cause congestion and overfilling in blood vessels.
Moreover, the radiological appearance of peliosis hepatis can also mimic many other diseases affecting the liver. The lesions of peliosis hepatis may vary greatly in size and location within the liver, sometimes leading to misdiagnosis as cancerous lesions. Different imaging techniques like angiography, triple-phase CT, or MRI are used to differentiate peliosis hepatis from other diseases. These imaging tests help reveal the pooling of blood in certain areas of the liver, a common characteristic of peliosis hepatis. However, these findings may vary depending on the severity of the condition and the extent of liver involvement.
The following conditions, seen through radiography, often bear similarities to peliosis hepatis and therefore form its differential diagnosis:
- Polycystic liver disease
- Congenital hepatic fibrosis
- Solitary congenital cysts
- Hydatid cysts
- Von Meyenburg complexes
- Caroli disease (type V choledochal cyst)
- Type IV choledochal cysts
What to expect with Peliosis Hepatis
The way in which peliosis hepatis, a rare liver condition, develops over time is still not well understood by scientists. There are no identifiable patterns indicating how the disease progresses. Some people who are diagnosed with this condition might not experience any symptoms for the rest of their lives, while others might develop serious issues associated with portal hypertension, a type of high blood pressure that affects the liver.
Very rarely, patients might also experience life-threatening complications, including internal bleeding in the abdomen, which is also known as intraperitoneal hemorrhage. These serious complications can lead to high mortality rates.
Possible Complications When Diagnosed with Peliosis Hepatis
While intraperitoneal (inside the abdominal cavity) and intrahepatic (inside the liver) bleeding appear to be uncommon after surgery, there is still a risk. Surgical removal of the affected areas can help prevent these complications, particularly if diagnostic images show the cavities getting worse.
Common Complications:
- Intraperitoneal bleeding
- Intrahepatic bleeding
Surgical removal can help avoid these complications, especially when the cavitary lesions (abnormal areas of tissue) are getting worse as seen in medical imaging tests.
Preventing Peliosis Hepatis
If you’ve been diagnosed with peliosis hepatis, it’s important your doctor or another healthcare provider gives you enough information about your condition and what to expect long-term. Peliosis hepatis is a rare condition where small blood-filled cysts form in your liver.
If these lesions were found unexpectedly during abdominal imaging and a biopsy showed this condition, you will likely be referred to a gastroenterologist. A gastroenterologist is a doctor with specialized knowledge about the digestive system, including the liver.
They will typically see you for regular check-ups each year to keep an eye on how your condition is progressing. Because there isn’t a lot of research on peliosis hepatis, a specialist should give you, your family, and your friends counseling early on and prepare you for what’s to come. This is done so that you can all better understand and manage your condition.
