Hepatosplenic T-cell lymphoma (HSTCL) is a rare but aggressive form of non-Hodgkin’s lymphoma most common in adolescents and young adults. Only about 200 cases exist in medical literature.

Lymphoma is a group of cancers that develop in a category of white blood cells called lymphocytes. Based on how the cancer cells appear under a microscope, doctors split lymphomas into two main categories: Hodgkin’s and non-Hodgkin’s lymphoma.

Experts expect more than 80,000 people in the United States to receive a non-Hodgkin’s lymphoma diagnosis in 2022, compared with about 8,540 for Hodgkin’s lymphoma.

About 5% to 15% of non-Hodgkin’s lymphomas are peripheral T-cell lymphomas. This type of cancer tends to be aggressive and develops in white blood cells called T cells and natural killer cells.

Less than 5% of peripheral T-cell lymphomas are HSTCL.

Understanding HSTCL

  • Hepatosplenic: The cancer develops either in your liver (“hepato”) or spleen (“splenic”).
  • T cell: These are a category of white blood cells that play an important role in your adaptive immune system, helping stop potential infections from developing. HSTCL develops in the T cells in your liver or spleen.
  • Lymphoma: This is a cancer that develops in lymphocytes. T cells are one of two main types of lymphocytes.

The development of lymphoma is still not well understood. Most people with HSTCL develop it with no known cause, but about 20% of the time, HSTCL develops in people with immunosuppression. It’s especially prevalent in people with:

Scientists have identified several genes that are at risk for developing the disease.

About 63% of people with HSTCL have a genetic mutation called isochromosome 7q. About 50% of people have an extra copy of chromosome 8 (trisomy 8).

HSTCL is more common in adolescents and young adults. Half of people who receive a diagnosis of HSTCL are under the age of 34 years old, and most people are under the age of 40 years old.

According to a 2020 study, males seem to be about five times more likely than females to develop HSTCL.

For people with IBD, the risk seems to be highest in those taking long-term thiopurines either alone or with anti-tumor necrosis factor agents.

The symptoms of HSTCL are similar to other lymphomas except for a lack of swollen lysies. Symptoms may include:

People with HSTCL also tend to develop “B symptoms.” These include:

Abdominal pain due to spleen or liver enlargement is common. If the cancer is advanced, jaundice may occur.

It can be difficult to identify the symptoms of HSTCL because they can mimic other diseases.

The process of diagnosing HSTCL is similar to other types of non-Hodgkin’s lymphoma. Doctors or healthcare professionals usually perform a liver or bone marrow biopsy to make a diagnosis. They’ll analyze cells from the biopsy in a laboratory using specialized tests such as flow cytometry.

If you have a problem with your liver or spleen, you may want to use a type of diagnostic equipment called a computed tomography (CT) Scan.

Researchers have limited knowledge about how to treat it. It is difficult to judge the effectiveness of various treatment options because of the small number of people treated at a single hospital.

Doctors treat people with the disease with a bone marrow transplant if they are eligible.

Researchers have looked into various chemotherapy regimens with varying levels of effectiveness. At least two small studies have investigated the CHOP regimen, which consists of the drugs:

  • cyclophosphamide
  • doxorubicin (hydroxydaunorubicin)
  • vincristine (Oncovin)
  • prednisone

Nine out of 21 people achieved complete remission, but half of them lived less than 16 months. Only two people out of six who were treated with CHOP achieved a full recovery. Remission lasted an average of 8 months.

The role of bone marrow transplants in treatment remains under investigation. Some studies have found evidence that they may improve outcomes.

A doctor may consider two types of bone marrow transplant:

  • Autologous transplant: Medical staff take stem cells from your own bone marrow and return them after you receive large doses of chemotherapy.
  • Allogeneic transplant: You receive stem cells from a donor who’s a close genetic match. The donor is often a relative.

In a 2022 study, researchers presented a case of a young person with HSTCL who doctors cured with a combination of an autologous and allogeneic stem cell transplant.

HSTCL tends to be aggressive, and the outlook for people with it tends to be poor. Estimates on the 5-year overall survival rate have been as low as 7%, with half of people living less than 10 months.

In a 2015 Dutch study, only 2 out of 12 people who receive a diagnosis of HSTCL lived for more than a year.

Researchers are still looking at the best way to treat people with the disease. The survival rate will increase due to the rarity of the disease.

Many estimates of survival rates for HSTCL draw on research from decades ago. For example, in a 2021 study, researchers calculated the following overall survival rates:

Time frame Overall survival rate
1 year 56.9%
3 years 37.6%
5 years 31.6%

The estimates were drawn from a group of 123 people who received a diagnosis of the disease.

“The outlook for people with this type of non-Hodgkin’s lymphoma is poor. T cells are a type of white blood cell that are found in the body.”

Doctors are looking at how to best treat the disease. The survival rate will likely improve as researchers improve their understanding of the disease.

Talk with a doctor about whether you’re eligible for clinical trials that may give you access to state-of-the-art treatments. You can also find a list of current trials on the U.S. National Library of Medicine website.