Hepatosplenic T-cell lymphoma
Hepatosplenic T-cell lymphoma (HSTL, or HSTCL) is a very rare T-cell lymphoma. It may also be called hepatosplenic gamma delta T-cell lymphoma.
HSTL is a very fast-growing (aggressive) type of NHL. The average age at diagnosis is 35. It develops in men more often than in women. It sometimes develops in people whose immune system isn’t working properly (suppressed). This includes people who have used immunosuppressive medicines for an autoimmune disease or after a solid organ transplant.
HSTL usually affects the liver or spleen. As a result, people with HSTL often have a larger than normal liver or spleen or both.
This type of NHL often affects the bone marrow, which can cause:
- low red blood cell counts (called anemia)
- low platelet counts (called thrombocytopenia)
HSTL can cause B symptoms, which are unexplained fever, drenching night sweats and unexplained weight loss.
People with HSTL may have skin sores. They usually don’t have larger than normal lymph nodes.
HSTL is very rare, so there is no standard treatment. You may be offered one or more of the following treatments.
Most often, people with HSTL are treated with a combination of chemotherapy drugs, such as:
- CHOP – cyclophosphamide (Cytoxan, Procytox), doxorubicin (Adriamycin), vincristine (Oncovin) and prednisone
- CVIDD/MTX/Ara-C – cyclophosphamide, vincristine, liposomal doxorubicin (Myocet), dexamethasone (Decadron, Dexasone), methotrexate and cytarabine (Cytosar, Ara-C)
Stem cell transplant
Some people with HSTL may be offered a stem cell transplant after chemotherapy.