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
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.
- 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
- There are disorders of the immune system.
- IBD is a disease of the colon.
- Previous cancer.
- A solid organ transplant is a good option.
Scientists have identified several genes that are at risk for developing the disease.
According to a 2020 study, males seem to be about
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 “
- There is a high degree of fever.
- drenching night sweats
- unintentional loss of more than 10% of your body weight over 6 months
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
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
- doxorubicin (hydroxydaunorubicin)
- vincristine (Oncovin)
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
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.
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|
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.