Medical Information
Hepatosplenic T-Cell Lymphoma

Hepatosplenic T-Cell Lymphoma

Digestive Health
Diseases & Conditions
Contributed byMaulik P. Purohit MD MPHSep 25, 2018

What are the other Names for this Condition? (Also known as/Synonyms)

  • Hepatosplenic Gamma Delta T-Cell Lymphoma
  • HSTCL (Hepatosplenic T-Cell Lymphoma)
  • HSTCL, Non-Hodgkin Lymphoma Type

What is Hepatosplenic T-Cell Lymphoma? (Definition/Background Information)

  • Hepatosplenic T-Cell Lymphoma (HSTCL) is an extremely rare and aggressive type of ‘extranodal lymphoma’ observed in the spleen, liver, and bone marrow. Extranodal lymphomas develop outside of the lymph nodes and can be found in body tissues, like the skin, tonsils, brain, bowels, and bone
  • Lymphoma is a type of blood cancer stemming from uncontrollably dividing lymphocytes (type of white blood cells). There are two types of lymphomas:
    • Hodgkin Lymphoma
    • Non-Hodgkin Lymphoma
  • HSTCL is a type of non-Hodgkin lymphoma. They are differentiated by detecting the type of proteins expressed on the surface of the cancer cells, and by the way they look under a microscope, when examined by a pathologist
  • Lymphocytes are a class of white blood cells that are part of the lymphatic system. Lymphocytes are made in bone marrow, and can develop into either B-cells or T-cells. T-cells mature in the thymus; HSTCL arises from cancerous T-cells
  • Normally, lymphocytes help generate an immune response to infections. These cells can recognize a wide variety of foreign invaders. They can also remember them and respond accordingly, if the body is infected with the same bacteria/virus ever again
  • The lymphatic system is responsible for immunity to diseases. The organs of the lymphatic system are lymph nodes, bone marrow, the spleen, thymus, and tonsils. T-cells can travel from the thymus to other parts of the lymphatic system
  • T-cells are responsible for recognizing cells that are altered or infected, and it includes attempting to kill cells that are cancerous too

Hepatosplenic T-Cell Lymphoma may be associated with inflammatory bowel disease that has been treated with immunosuppressive medication, and other immunosuppressed conditions.

  • Common signs and symptoms of HSTCL are low red blood cell counts, low platelet counts, liver and spleen swelling, weight loss, and excessive night sweats
  • The condition is generally treated with a combination of chemotherapy, bone marrow transplants, and through removal of the spleen
  • However, the prognosis of HSTCL is very poor, with a high risk of relapse

Who gets Hepatosplenic T-Cell Lymphoma? (Age and Sex Distribution)

  • Hepatosplenic T-Cell Lymphoma is an extremely rare condition. It accounts for less than 1% of all non-Hodgkin lymphoma cases (in the US), and only 1.4% of T-cell lymphoma cases
  • The condition is most common in adolescents  and young adults (median age 35 years, at the time of diagnosis)
  • Males are much more likely to develop HSTCL, than females
  • There is no specific ethnic or racial predisposition

What are the Risk Factors for Hepatosplenic T-Cell Lymphoma? (Predisposing Factors)

Some of the risk factors associated with Hepatosplenic T-Cell Lymphoma include:

  • Transplant patients on immunosuppressive medication
  • Treatment of inflammatory bowel diseases (especially Crohn’s disease) with immunosuppressive medications, infliximab, and tumor necrosis factor alpha-blocking agent
  • Exposure to chemicals, such as pesticides and fertilizers, as well as smoking and diet, has been suggested as links to other forms of non-Hodgkin lymphoma.
  • Epstein-Barr virus (EBV) has been associated with some non-Hodgkin lymphoma cases; but, those diagnosed with HSTCL, are usually EBV negative

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Hepatosplenic T-Cell Lymphoma? (Etiology)

Currently, the exact cause of Hepatosplenic T-Cell Lymphoma is not completely understood.

What are the Signs and Symptoms of Hepatosplenic T-Cell Lymphoma?

The signs and symptoms of Hepatosplenic T-Cell Lymphoma include:

  • Anemia (low red blood cell count)
  • Thrombocytopenia (low platelet count)
  • Autoimmune disorders, which can cause joint and muscle pain, heat intolerance, recurrent rashes, abdominal pain, and a general feeling of illness
  • Liver and spleen enlargement
  • Loss of appetite and weight loss
  • Fatigue
  • High temperatures and excessive night sweats
  • ‘Non-painful’ swelling of lymph nodes in the neck, armpits, or groin, may be present; though, this is an uncommon finding

Abnormal liver function tests, irregular lymphocytes in blood (atypical lymphocytes), and low neutrophils (circulating white blood cells), are some other indications.

How is Hepatosplenic T-Cell Lymphoma Diagnosed?

It often takes multiple tests to diagnose lymphoma. It is critical that the specific sub-type of lymphoma be diagnosed correctly, in order to create a proper treatment plan. Hepatosplenic T-Cell Lymphoma diagnosis involves:

  • A physical examination and a complete medical history by a doctor can help determine, if there is a risk of lymphoma
  • Bone marrow biopsies are often performed first, to determine if the bone marrow is involved by HSTCL
  • Biopsies of the liver or spleen
  • HSTCL usually does not involve the lymph nodes. Biopsies of lymph nodes that may be enlarged can be taken and examined in a lab, to determine if the cells are malignant or benign. The biopsies may be performed under general or local anesthesia. Normally, the entire lymph node is removed to help determine the sub-type of lymphoma
  • Blood tests to evaluate red and white blood cells, as well as platelet counts
  • Lumbar puncture to determine, if HSTCL involves the brain
  • X-rays of chest and abdomen, to look for enlarged lymph nodes, liver, or spleen
  • Whole body CT-PET scan - to determine the spread of lymphoma, by determining the size and metabolic rate (a reflection of uncontrolled growth) of lymph nodes, throughout the body. This can also determine if the cancer has spread to other organ systems
  • Brain MRIs are used if neurologic symptoms are present, to determine if cancer has spread to the brain, or to the tissue that covers the brain (meninges)

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Hepatosplenic T-Cell Lymphoma?

The possible complications from Hepatosplenic T-Cell Lymphoma include:

  • HSTCL is extremely aggressive and can spread to other locations
  • Treatments can cause secondary health problems, such as secondary forms of cancer and heart disease
  • There is a high risk of relapse and a low percent of long-term survival

How is Hepatosplenic T-Cell Lymphoma Treated?

Once a definitive diagnosis of Hepatosplenic T-Cell Lymphoma has been made, staging is performed to determine how far the cancer has spread. The stage can describe:

  • How many lymph nodes are affected (if any)
  • Their locations in the body
  • And, if other organs are being affected

Staging is important because different treatment regimens are necessary, depending on the progression of the lymphoma. Even though Hepatosplenic T-Cell Lymphoma does not usually include lymphadenopathies (enlarged lymph nodes), these stages can still be used to describe cancer progression.

  • Stage 1: Only one group of lymph nodes in one location is affected
  • Stage 2: Lymph nodes in multiple locations that are all above or below the diaphragm are affected
  • Stage 3: Lymph nodes in multiple locations, both above and below the diaphragm are affected
  • Stage 4:  Lymph nodes located throughout the body, the bone marrow, and other organs are affected
  • Category A: No symptoms are present at the time of diagnosis
  • Category B: Presence of one or more symptoms at the time of diagnosis

Designation of extranodal sites:

  • Marrow = M+
  • Lung = L+
  • Liver = H+
  • Pleura = P+
  • Bone = O+
  • Skin and subcutaneous tissue = D+

A combination of treatments is used to most effectively treat this cancer. The aggressiveness of the treatment is determined by the disease progression.

Surgery: After initial biopsies to diagnose HSTCL, surgical removal of the spleen (splenectomy) may help relieve some symptoms. However, this will not cure patients of the condition.

Chemotherapy: This approach uses a combination of drugs to kill the cancerous cells and can be used for patients, at all stages of HSTCL.

  • Commonly, the drug combination CHOP is used first, which includes doxorubicin, vincristine, cyclophosphamide, and prednisolone
  • There can be severe side effects including fatigue, nausea, hair loss, anemia, high risk of infection, and drug-specific reactions
  • Many T-cell lymphomas can be resistant to chemotherapy. It can also damage healthy cells
  • Chemotherapy can be administered as a pill, liquid, shot, or intravenously. Although, this may work initially, and then stop being effective

Radiation: Radiation therapy is the use of high-energy radiation waves, to kill cancer cells by destroying their DNA.

  • This treatment modality is generally used for early stage lymphomas. It is most commonly used in combination with chemotherapy
  • The radiation may be administered by a machine placed outside the body, or by putting a radioactive material inside the body
  • The side effects of radiation therapy include nausea, vomiting, fatigue, pain, risk of cancer later in life, and a risk of heart disease
  • Radiation can damage healthy cells in addition to cancer cells, causing further complications

Bone marrow transplant: Blood stem cell transplants from a compatible donor are often used to help treat Hepatosplenic T-Cell Lymphoma. The patient’s own blood stem cells, harvested before treatment, cannot be used because of a high incidence of bone marrow involvement in HSTCL.

Supportive treatment: Steroids, blood transfusions, anti-nausea medications, and antibiotics, may also be used. In combination with other treatment measures, these can help combat the symptoms of immune deficiency.

Clinical trials: There are some new treatment options, currently on clinical trials that can be considered for some patients, depending on their risk factors.

How can Hepatosplenic T-Cell Lymphoma be Prevented?

  • Currently, there are no definitive ways to prevent Hepatosplenic T-Cell Lymphoma
  • However, healthy diet and exercise, as well as avoidance of unnecessary exposure to chemicals, may help decrease its risk
  • Regular medical screening at periodic intervals with blood tests, scans, and physical examinations, are mandatory for those who have already endured the tumor, due to its metastasizing potential and possibility of recurrence. Often several years of active vigilance is necessary

What is the Prognosis of Hepatosplenic T-Cell Lymphoma? (Outcomes/Resolutions)

  • Hepatosplenic T-Cell Lymphoma is an aggressive form of T-cell lymphoma, with a high risk of relapse. The prognosis of HSTCL is generally poor
  • There is an average survival of less than 2 years for individuals with the condition

Additional and Relevant Useful Information for Hepatosplenic T-Cell Lymphoma:

  • There are fewer than 100 people in reported literature, who have been diagnosed with Hepatosplenic T-Cell Lymphoma
  • T-cell lymphomas are less common than B-cell lymphomas
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Maulik P. Purohit MD MPH picture
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Maulik P. Purohit MD MPH

Assistant Medical Director, Medical Editorial Board, DoveMed Team


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