Although lymphoma is categorized as a type of blood cancer, it can cause cancer in many other organs, including your liver. Primary hepatic lymphoma (PHL) is a rare type of non-Hodgkin lymphoma (NHL) that involves the liver.
Lymphoma is a cancer that starts in white blood cells called lymphocytes and affects your lymphatic system. Your lymphatic system is part of your immune system (which helps keep you healthy and fight infections) and primarily consists of your bone marrow, thymus, spleen, and lymph nodes. However, you have a network of lymphatic vessels and tissue all through your body.
When lymphoma starts in the lymphatic tissue of your liver, it’s called primary hepatic lymphoma. Continue reading to learn more about PHL.
Primary hepatic lymphoma is a type of extranodal lymphoma — it starts outside the lymph nodes. As its name suggests, PHL is a type of lymphoma that affects the liver. It may also be referred to as primary non-Hodgkin lymphoma of the liver.
While it’s common for other types of lymphoma to have liver involvement or spread to your liver (known as metastasis), it’s very rare for lymphoma to start in your liver.
The type of lymphoma causing PHL is determined by a pathologist — a doctor who specializes in examining body tissues.
The most common subtype of lymphoma is diffuse large B-cell lymphoma (DLBCL). Between 46 percent and 68 percent of primary hepatic lymphoma cases are caused by DLBCL. Less commonly, PHL can be caused by other types of lymphoma, including:
Primary hepatic lymphoma is very rare, making up just 0.4 percent of extranodal NHL cases and 0.016 percent of all cases of NHL.
PHL occurs more often in males than females, according to StatPearls, a clinical support tool. The condition usually shows up just over age 50, but diagnoses have been made in people ranging in age from the 20s to the 80s.
Researchers don’t know exactly what causes primary hepatic lymphoma. However, other medical conditions have been associated with an increased risk of developing PHL.
People infected with viruses that affect the liver, such as the hepatitis C virus and hepatitis B virus, may have an increased risk of developing PHL. Other viruses that don’t infect the liver have also been linked to PHL, such as the human immunodeficiency virus and cytomegalovirus.
According to StatPearls, about 10 percent of people diagnosed with PHL already have another type of liver disease. These conditions include cirrhosis (permanent liver scarring) and chronic hepatitis (long-term liver inflammation).
In a 2016 retrospective study of 41 people with PHL, researchers reported that almost 10 percent of cases were diagnosed in people who underwent a liver transplant.
Autoimmune disorders, such as systemic lupus erythematosus, can also cause liver damage that may raise your risk of PHL. People taking immunosuppressant drugs after receiving an organ transplant may be at increased risk.
Most people with primary hepatic lymphoma have vague, nonspecific symptoms that could be caused by other conditions. The most common symptoms include:
People with PHL may also experience B symptoms of lymphoma — fever, night sweats, and weight loss. The presence or absence of these symptoms plays a role in the stage and outlook of the disease.
PHL can also cause liver problems. Sometimes this organ gets bigger, which is called hepatomegaly. You might feel a lump in your abdomen and have abdominal pain on the right side. In rare cases, PHL may also cause chronic hepatitis or acute hepatic failure (the liver suddenly stops functioning).
Notably, lymphadenopathy (swollen lymph nodes), a common symptom of other types of NHL, isn’t typical with PHL.
It can be difficult to diagnose primary hepatic lymphoma because the condition is so rare and the symptoms are so general.
Your care team will use several tests to help diagnose PHL. They may also first eliminate other potential causes, known as a differential diagnosis. The lesions (damaged tissue) caused by PHL can look like other types of lesions, such as hepatocellular carcinoma, metastatic liver disease, or a liver abscess.
The only way to confirm a PHL diagnosis is with a biopsy — your doctor takes a sample of cancer cells from the tumor to be examined under a microscope.
Your doctor may order blood work to check for signs of PHL. One type of blood test is the complete blood count, which measures the amounts of different types of cells in your blood. Unlike people with other types of NHL, a person with PHL may have blood counts in the normal range.
Your doctor will also check the amount of substances in your blood that your liver releases — these are known as liver function tests. People with PHL may have higher-than-normal levels of substances such as:
To help make a diagnosis, your doctor may test for other substances in your blood called tumor markers, such as:
To get a better look at the tumor caused by PHL, your doctor can use imaging tests, such as:
The liver lesions caused by PHL may show up as a solitary lesion, multiple lesions in the liver, or a diffuse infiltration (found throughout the entire liver).
Because primary hepatic lymphoma is a very rare type of lymphoma, the best treatment is unclear. Options include surgery, chemotherapy, radiotherapy, or a combination of treatments.
People with localized disease may have a surgical resection (removal) of the tumor. Surgery may also be performed before chemotherapy.
Chemotherapy is the recommended treatment for people with DLBCL. Some people may be able to be treated with chemotherapy only. A common chemotherapy regimen used to treat PHL is called CHOP, which consists of four chemotherapy drugs:
People with DLBCL may also benefit from using a targeted therapy called rituximab (Rituxan) along with their chemotherapy regimen. When rituximab is added to CHOP, it’s referred to as R-CHOP.
In general, people diagnosed with PHL have a more favorable outlook than people who have other types of liver cancer. Primary hepatic lymphoma usually responds well to treatments like chemotherapy. Signs and symptoms can disappear completely — also called complete remission — in up to 80 percent of people diagnosed with PHL, according to StatPearls.
Factors associated with a worse prognosis (outlook) include:
The prognosis for PHL can be measured by median survival time — the length of time that half the people diagnosed with PHL are expected to still be alive. In the 2016 retrospective study, researchers reported a median survival of 163 months (about 13.5 years). They found that 77 percent of people were still alive five years after diagnosis (five-year survival) and 59 percent were still alive after 10 years (10-year survival).
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