Lymphomas
Hepatosplenic T-cell lymphoma (HSTCL)
If you or someone you love has just heard this diagnosis, start here. This guide explains what the condition is, how it is usually treated and where a transplant fits.
Hepatosplenic T-cell lymphoma is a rare, aggressive lymphoma that mainly involves the liver, spleen and bone marrow. Early specialist treatment and donor-transplant assessment are important because ordinary lymphoma chemotherapy alone often gives short-lived control.
Other names and abbreviations
HSTCL, HSTL, hepatosplenic γδ T-cell lymphoma, hepatosplenic αβ T-cell lymphoma, Hepatosplenic gamma-delta T-cell lymphoma, hepatosplenic alpha-beta T-cell lymphoma
In short
- Hepatosplenic T-cell lymphoma is a rare, fast-growing lymphoma. It mainly affects the liver, spleen and bone marrow.
- It is usually treated with strong drug combinations. That is because ordinary lymphoma chemotherapy alone often controls it only for a short time.
- For eligible patients, doctors often look early at a donor transplant. That is because donor cells can help fight the lymphoma, though a cure is not guaranteed.
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Underlined words open a short explanation. See all terms
Where transplant fits
Eligible patients are often assessed early for allogeneic transplantationComing from another person. In an allogeneic, or donor, transplant, the stem cells come from a relative or an unrelated volunteer whose cells are a close enough match to the patient's., including during first-line treatment. Donors can provide an immune effect against this aggressive lymphoma, but the evidence is limited and neither remissionA decrease in or disappearance of the signs of a disease. In complete remission, no signs can be found, but some disease cells may still be in the body. nor cure is guaranteed.
Treatment depends on the exact diagnosis, disease stage, prior treatment and the person’s health.
Some patients need a donor who is not a relative.
See if you can joinKey facts
- Who it affects
- This rare lymphoma affects males more often than females. It has mainly been described in adolescents and younger adults, but a recent study cited in the WHO classification found about half of patients were 60 or older. Some cases are associated with immune dysregulation or immunosuppressive treatment.
- How common
- About 1% of peripheral T-cell lymphomasShare of peripheral T-cell lymphoma cases, NCI PDQ summary of published studies (updated May 2025) Source: How common
- Cells used in a transplant
- Donated blood-forming cells for allogeneic transplantation. Marrow, peripheral blood or cord blood and donor type are selected for the patient and transplant approach.
- Where a donor fits
- Donor transplant option
The condition
What it is
This lymphoma grows in small blood-filled channels in the liver, spleen and marrowThe soft, spongy tissue in the center of most bones. Red bone marrow holds the blood-forming stem cells that make red blood cells, white blood cells and platelets.. It often lacks the prominent enlarged lymph nodes seen with other lymphomas. Most cases arise from gamma-delta T cellsA type of white blood cell that is part of the immune system. T cells grow from stem cells in the bone marrow, help protect the body from infection and may help fight cancer., although an alpha-beta form also exists.
It affects males more often than females. It has long been described as a disease of younger people, but in a study cited by the World Health Organization in 2022, about half of the people with it were 60 or older. Diagnosis requires specialist review of marrow or tissue and immune-marker findings. An enlarged spleen alone does not identify this disease.
Marked as affected: T cells.
- Blood stem cell, In the bone marrow
- Myeloid line
- Red blood cells
- Platelets
- Granulocytes
- Monocytes
- Lymphoid line
- B cells
- Plasma cells, Develop from B cells
- T cells, Affected
- NK cells, Natural killer cells
- Myeloid line
What causes it
The lymphoma cells acquire changes that disrupt growth and survival. Some cases occur in people with immune dysregulation or long-term immunosuppressive treatmentTreatment that lowers the activity of the immune system. It can calm an immune attack on the body, as in aplastic anemia, or help keep the body from rejecting a transplant. It also makes infections harder to fight., while others have no such history.
These associations do not establish that one medicine caused an individual case. Decisions about medicines for an underlying immune condition are made with the doctors who treat that condition. Hepatosplenic T-cell lymphoma is not contagious.
Symptoms and effects
Symptoms can include fever, night sweats, weight loss and abdominal fullness from a large spleen or liver. Low plateletsTiny pieces of cells in the blood that help form clots to slow or stop bleeding. They are made in the bone marrow. Too few platelets can cause easy bruising and bleeding., anemia and other blood-count abnormalities can be substantial.
The disease can progress quickly. Infection, bleeding, liver dysfunction and inflammatory complications may add to the burden, so supportive care and treatment planning often need to proceed together.
A simple drawing of a body. Often affected: liver, spleen and bone marrow.
Often affected
- Liver: large liver
- Spleen: large spleen
- Bone marrow
This shows the parts of the body the lymphoma most often affects, not every place it can reach, and it says nothing about how severe it will be.
Diagnosis and treatment
How hepatosplenic T-cell lymphoma is diagnosed
This lymphoma is hard to recognize. It usually does not cause the swollen lymph nodes seen in many other lymphomas. Its first signs, such as fever, a large liver and spleen, and low blood counts, can look like an infection or another cancer. For these reasons, the diagnosis is often delayed.
Today the diagnosis is usually made from a bone marrow biopsy, a liver biopsy or both. In the past, removing the spleen was the most common way to reach it; that is now rarely needed. In the marrow, lymphoma cells grow inside small blood channels (sinusoids). Tests of the markers on the cells (immunophenotyping) show T cells. In most cases they carry the gamma-delta T-cell receptor.
A chromosome test (cytogenetics) often finds an abnormal chromosome made of two copies of the long arm of chromosome 7 (isochromosome 7q), an extra chromosome 8 (trisomy 8), or both. These changes support the diagnosis. Scans usually show a large liver and spleen without separate lumps. On a PET-CT scan, the liver, spleen and marrow may light up evenly.
Under the microscope, the marrow cells can look like those seen in myelodysplastic syndrome (MDS), a different marrow disorder.
How blood counts can look in hepatosplenic T-cell lymphoma (HSTCL): red blood cells low and platelets low.
- Red blood cells: Low. Anemia.
- Platelets: Low
Each lab has its own usual range, and it changes with age. The drawing shows only the direction a count often moves, not how far, and one person’s results can look different.
How it is treated
Treatment commonly uses intensive combinations containing medicines such as cytarabine, ifosfamide or platinum drugs. Standard CHOP chemotherapy alone has often produced inadequate durable control. Clinical trialsA research study that tests how well a new medical approach works in people. Trials can test new ways to screen for, prevent, diagnose or treat a disease. and care at a center experienced with rare T-cell lymphomas are relevant.
Allogeneic transplantation is often considered early for eligible patients, including as part of first-line treatment after disease control. The aim is to combine treatment of the lymphoma with an immune effect from donor cells.
The evidence base is limited by the disease’s rarity and largely comes from small series and registry studies. Transplant can produce long-term remission, but relapseWhen a disease comes back after a period of getting better. Relapsed disease has returned after treatment helped for a time. and serious transplant complications remain possible; it is not a guaranteed cure.
Kinds of treatment described for hepatosplenic T-cell lymphoma (HSTCL): supportive care, medicines, a donor stem cell transplant and clinical trials.
After diagnosis, the options described here
Supportive care
Transfusions, infection prevention and care for belly discomfort may be needed during treatment.
Medicines
Strong drug combinations are used, because ordinary lymphoma chemotherapy alone often controls it only for a short time.
Donor stem cell transplant
A donor transplant is often considered early for people who are eligible, because donor cells can help fight the lymphoma.
What a transplant involvesClinical trials
Clinical trials and care at a center experienced with rare T-cell lymphomas can matter.
These are the kinds of treatment this page describes, not a plan. Which ones fit, in what order and whether they are combined differs from person to person.
When transplant specialists are usually consulted
The NMDP and ASTCT consultation timing guidelines list mature T-cell and NK-cell lymphomas, which include this one, for a transplant consultation at diagnosis or in first remission, and again at relapse. NMDP notes that when a donor transplant may be needed, HLA typing at diagnosis is a key first step toward finding a donor.
Read the guidanceWhat a transplant involves
- Step 1
: Finding a donor
Relatives are tested first to see whether their tissue type (HLA) matches. If none match, the team searches donor registries and cord blood banks.
- Step 2
: Conditioning
Chemotherapy, sometimes with radiation, prepares the body for the new cells.
- Step 3
: Transplant day, Day 0
The donor’s cells are given through a vein, like a transfusion.
- Step 4
: Engraftment
The new cells settle in the marrow and start making blood cells, usually within weeks.
- Step 5
: Recovery
The immune system rebuilds over months. The team watches for infection, graft-versus-host disease (donor immune cells attacking the body) and relapse.
Daily life and the donor’s role
Living with the condition and treatment
The speed of the disease may leave little time between diagnosis, treatment and transplantA treatment that gives a patient healthy blood-forming stem cells through a vein. The cells travel to the bone marrow and replace faulty marrow or marrow damaged by treatment. They can come from the patient or a donor. assessment. Families may need help with travel, work and arrangements for care near a specialist center.
TransfusionsPutting blood, or parts of blood such as red cells or platelets, into a person's bloodstream through a vein. Some people with blood disorders need regular transfusions., infection prevention and management of abdominal discomfort may be needed during treatment. Following transplantation, recovery includes repeated laboratory testing and monitoring for infection, graft-versus-host diseaseA complication of a donor transplant. The donated cells see the patient's healthy tissues as foreign and attack them, especially the skin, liver and gut. It can start soon after transplant or much later and can be life-threatening. and recurrence.
The role of a blood stem cell donor
A donor can be central to treatment when allogeneic transplantation is planned. HLA typingA lab test that finds a person's tissue type (HLA markers). It starts with a blood draw or a cheek swab. Doctors compare a patient's results with those of relatives, registry donors and cord blood units. and a search can begin early while initial treatment is underway.
A suitable unrelated registry donor may be used, as may a relative or another appropriate graft sourceThe blood-forming stem cells given to a patient in a transplant. In a donor transplant, the graft comes from the donor's bone marrow or blood, or from donated cord blood.. The team balances donor suitability and availability against disease urgency. More registry volunteers mean more possible donors, but a donor cannot decide how any one person’s lymphoma will respond.
Highlighted here: a relative, an unrelated volunteer and donated cord blood.
The person’s own cells
Autologous transplant, no donor
Collected from the person before treatment, then given back.
A relative
Donor transplant (allogeneic)
A brother or sister may be a full match. Parents and children can be half-matched donors.
An unrelated volunteer
Donor transplant (allogeneic)
Found through a donor registry.
Donated cord blood
Donor transplant (allogeneic)
Collected from a baby’s umbilical cord after birth and stored in a public bank.
Some patients rely on a volunteer donor they have never met. Joining your country’s registry could make you that person for someone.
Join the registryFinding a donor and the outlook
How a donor is found
When a transplant from a donor is planned, the team usually tests brothers and sisters first. Each full sibling has about a one in four chance of being a full match.
Most patients do not have a matched relative. In the words of NMDP, the U.S. registry, “75% of patients don’t have a fully matched donor in their own family.” The team then searches registries of volunteer donors around the world and banks of donated cord blood. In some transplants, a half-matched parent, child or sibling can also be the donor.
8 of 8
All eight markers match. Doctors call this a full match.
8 of 8: the donor matches the patient at all eight markers, two each for HLA-A, HLA-B, HLA-C and HLA-DRB1.
7 of 8
One marker differs. Some transplants use a donor like this.
7 of 8: the donor matches at seven of the eight markers. One HLA-C marker differs.
Half-matched
One set, inherited together from one parent, matches. The rest may or may not.
Half-matched: the donor matches the four markers the patient inherited from one parent. The other four may or may not match.
- Matches
- Differs
- May or may not match
- Top row: from one parent. Bottom row: from the other.
- DR means HLA-DRB1
Doctors can look at up to 12 HLA markers, and usually aim to match 8 to 10 of them. This drawing shows the 8 that transplant guidelines count, and it reads each one as simply matching or not.
Matching depends on inherited tissue markers called HLA, so a patient is most likely to match someone who shares their ancestry. Every person who joins makes the search a little more likely to succeed, especially for patients from groups that are underrepresented on registries.
Looking ahead
Outlook for hepatosplenic T-cell lymphoma
Hepatosplenic T-cell lymphoma is one of the hardest lymphomas to treat. Standard CHOP chemotherapy often works only for a short time. A 2020 review in the journal Blood reports that, without a transplant, fewer than 1 in 10 people are alive after 5 years.
Based on limited data, experts favor strong chemotherapy other than CHOP, then a transplant once the lymphoma is under control. In a pooled analysis of 118 people, regimens other than CHOP were linked to longer survival. A registry study followed 94 adults in Europe and Asia who had a donor (allogeneic) transplant from 2005 to 2024. Of them, 55% were alive 3 years later.
Outlook also depends on how well the lymphoma responds to treatment. In one U.S. center’s series, not reaching a complete remission was linked with a worse outlook. The figures below come from people treated in the past, some many years ago. They cannot predict what will happen to one person.
About these numbers. Each one says which group of people it comes from, and the place and years where the source gives them. It describes what happened across that group, not what will happen to any one person. And a figure measured among people who had a transplant is not the same as the number of people who need one.
- 31.6%5-year overall survival, all people in the study
123 people diagnosed 1975–2016, U.S. SEER 18 registry areas, all treatments
Read the source: 5-year overall survival, all people in the study - 55%3-year overall survival after a donor (allogeneic) transplant
94 adults transplanted December 2005 to January 2024 at EBMT and cooperating Asian centers in Europe and Asia
Read the source: 3-year overall survival after a donor (allogeneic) transplant
Estimates differ between studies. The U.S. registry figure counts everyone diagnosed, whatever treatment they had. The transplant figure describes only adults who were able to have a transplant, not everyone diagnosed.
Common questions
What is the survival rate for hepatosplenic T-cell lymphoma?
It is lower than for most lymphomas. A 2020 review in Blood reports that without a transplant, fewer than 10% of people are alive after 5 years. People who are able to have a donor transplant do better. The outlook section on this page gives the figures, with the groups they describe. These numbers cannot predict one person’s outcome.
Is hepatosplenic T-cell lymphoma linked to Crohn’s disease medicines?
There is a reported link. A 2020 review in Blood says about 20% of cases arise in people with long-term immune suppression or an immune system disorder. By then, more than 30 cases had been reported in people with inflammatory bowel disease (Crohn’s disease or ulcerative colitis). Medicines linked to these cases include thiopurines (azathioprine or 6-mercaptopurine) and anti-TNF medicines such as infliximab, especially when taken together. In one series of 14 young people with both conditions, all had taken a thiopurine. The lymphoma is still very rare, and these reports cannot show what caused any one person’s illness.
Is hepatosplenic T-cell lymphoma curable?
It can be for some people, most often after a donor stem cell transplant. A common plan is strong chemotherapy other than CHOP, then an allogeneic transplant once the lymphoma is under control. U.S. (ASTCT) and European (EBMT) transplant guidelines both recommend a donor transplant as part of first treatment. In a 2026 registry study of 94 adults, 50.5% were alive without lymphoma 3 years after a donor transplant. The main risk is the lymphoma coming back: 37.9% relapsed within 3 years. A transplant with a person’s own cells was often followed by relapse in the same study.
Can children get hepatosplenic T-cell lymphoma?
Yes, but it is exceptionally rare in children. U.S. registry data from 2001 to 2012 found about 0.05 cases per million people under 21 each year. Some young people with inflammatory bowel disease have developed it. There is no standard treatment for children, so they are often treated like adults: intensive chemotherapy, then a donor transplant. A 2025 review estimates 5-year survival for children and teens at below 15%. This estimate comes from very small numbers and cannot predict one child’s outcome.
Why is hepatosplenic T-cell lymphoma hard to diagnose?
It does not usually cause swollen lymph nodes. Instead, it grows inside the liver, spleen and bone marrow. Its first signs, such as fever, a large spleen and low blood counts, can look like an infection or another cancer, so the diagnosis is often delayed. It is confirmed with a marrow or liver biopsy, tests of the markers on the lymphoma cells and chromosome tests.
For your next appointment
Hepatosplenic T-cell lymphoma (HSTCL)
From the Jada Bascom Foundation disease library, jadabascomfoundation.org. Printed .
Questions to bring to your care team
- Has a lymphoma pathology expert confirmed the diagnosis, including the T-cell receptor type and the chromosome tests?
- Can my brothers and sisters have HLA typing now, and when will an unrelated donor search start?
- Which first treatment is planned instead of standard CHOP, and is there a clinical trial for this lymphoma?
- Before intensive treatment begins, is there time to talk about protecting fertility?
- What is the exact name of the diagnosis or subtype, and what does it mean for treatment?
- What is the goal of each treatment you are suggesting?
- Is a transplant being considered? Why now, or why not yet?
- What happens if a fully matched donor is not found?
- Where can our family find support during treatment?
A one-page list to take to the next appointment, with room for notes.
Supporting someone with a diagnosisSupport for patients and families
These independent organizations offer information and support. JBF is not affiliated with them.
- Lymphoma Action UK charity with a freephone helpline, live chat, monthly support meetings, peer buddies and free books for anyone affected by lymphoma.United Kingdom
- Lymphoma Research Foundation US nonprofit devoted to lymphoma, offering a helpline, a free T-cell lymphoma guide, peer support and financial support resources for patients and caregivers.United States
Sources and further reading
- Peripheral T-Cell Non-Hodgkin Lymphoma Treatment (PDQ), Health Professional Version
NCI, Accessed 2026-09-05 - Other B- and T-Aggressive Lymphomas and Lymphomas Associated with HIV
EBMT Handbook / NCBI Bookshelf, Accessed 2026-09-05 - Indications for haematopoietic cell transplantation and CAR-T: 2025 EBMT practice recommendations
EBMT / Bone Marrow Transplantation, Accessed 2026-09-05 - WHO fifth-edition classification: Lymphoid Neoplasms
WHO classification authors / Leukemia, Accessed 2026-09-05 - Stem Cell and Bone Marrow Transplants for Cancer
NCI, Accessed 2026-09-05 - Donor and cord blood unit selection guidelines
NMDP / CIBMTR, Accessed 2026-09-05 - Haematopoietic stem cell transplantation in hepatosplenic T-cell lymphoma: a retrospective analysis of the EBMT Lymphoma Working Party
The Lancet Haematology (EBMT Lymphoma Working Party, via Europe PMC), 2026-08-13 (online); accessed 2026-09-26 - Join the registry
NMDP, Accessed 2026-09-24 - On modeling human leukocyte antigen-identical sibling match probability for allogeneic hematopoietic cell transplantation
Biology of Blood and Marrow Transplantation, March 2016 - Allogeneic Hematopoietic Cell Donor Selection: Contemporary Guidelines from the NMDP/CIBMTR
NMDP / CIBMTR, Transplantation and Cellular Therapy, 2025 - What is HLA? HLA basics, typing and matching
NMDP, Accessed 2026-09-26 - Matching with a patient
NMDP, Accessed 2026-09-26 - Hepatosplenic T-cell lymphoma: a rare but challenging entity
Blood (Pro, Allen and Behdad, review), via PubMed Central, 2020-08-05 (online); accessed 2026-09-26 - Hepatosplenic T-cell lymphoma in children and adolescents
Blood Advances (Schwartz, Devine and Xavier, review), via PubMed Central, 2025-04-22; accessed 2026-09-26 - Survival Analysis of Hepatosplenic T Cell Lymphoma: A Population-Based Study Using SEER
International Journal of General Medicine (Li et al., SEER analysis), via PubMed Central, 2021-11-17; accessed 2026-09-26 - Clinical Practice Recommendations on Indication and Timing of Hematopoietic Cell Transplantation in Mature T Cell and NK/T Cell Lymphomas
ASBMT (now ASTCT) Guidelines Committee, Biology of Blood and Marrow Transplantation (abstract via Europe PMC), 2017-08-07; accessed 2026-09-26 - HCT consultation timing guidelines: Non-Hodgkin lymphoma (NHL)
NMDP / ASTCT, Accessed 2026-09-26 - HCT consultation timing guidelines & outcomes: overview
NMDP, Accessed 2026-09-26
This information explains a condition and its treatments. It cannot diagnose an illness or recommend treatment for an individual. Your care team can explain how the evidence applies to you. Written and source-checked by the Jada Bascom Foundation. Each page lists the published sources it draws on.
Ways to help
Someone may be waiting for a match.
Some people with hepatosplenic T-cell lymphoma (HSTCL) are treated with a transplant from a donor. When no relative matches, that donor is often a stranger who joined a registry.
Join the registry
JBF points you to the official registry that serves your country. It explains who can join and what donation involves.
Help someone you love find a donor
If someone you love needs a donor, our family guide explains practical ways to help. A registration drive can add many potential donors at once, for them and for others.
Support this work
Gifts to the Jada Bascom Foundation support donor-awareness education like this page, community outreach, drive planning and referrals to official registries.
More in the library
Keep learning
Part of 4 diagnosis guides, each explaining how its subtypes fit together: T-cell lymphoma, Non-Hodgkin lymphoma (NHL), Lymphoma and Types of blood cancer.
