Hepatosplenic T-cell lymphoma is a rare type of high-grade non-Hodgkin lymphoma that grows in the liver and spleen.
It usually affects younger adults, typically people in their mid-30s.
It is more common in men than women.
Hepatosplenic T-cell lymphoma is linked with immune deficiency: more common in patients on medicines that suppress the immune system – after a transplant or for inflammatory bowel diseases such as Crohn’s disease.
It can occur spontaneously in people who don’t have pre-existing conditions and aren’t taking medication.
Common problems of hepatosplenic T-cell lymphoma are: hepatomegaly,
ascites, jaundice, splenomegaly,
anemia, thrombocytopenia and
neutropenia, and weight loss.
It is mot associated with lymphadenopathy.
Most people with hepatosplenic T-cell lymphoma have advanced stage lymphoma at diagnosis.
No treatments have become established as recognized standard of care for hepatosplenic T-cell lymphoma.
The most common chemotherapy regimens used for hepatosplenic T-cell lymphoma are those used for aggressive lymphomas.
HSCT are considered for responders.
Relapsed or refractory hepatosplenic T-cell lymphoma is common.