What is Hepatosplenic T-Cell Lymphoma?
Hepatosplenic T-cell lymphoma (HSTCL) is a rare and aggressive form of peripheral T-cell lymphoma. It is characterized by the abnormal proliferation of T-cells, a type of white blood cell, in the liver and spleen. This disease primarily affects young adults, with a higher prevalence in males. Patients with HSTCL often present with symptoms such as fever, weight loss, and abdominal pain due to the enlargement of the liver and spleen. The disease is often advanced at the time of diagnosis, with involvement of the bone marrow and peripheral blood.
Why do people get Hepatosplenic T-Cell Lymphoma?
The exact cause of Hepatosplenic T-Cell Lymphoma is not known. However, it is believed to be associated with certain risk factors. Some of these include a history of immunosuppression, such as in organ transplant recipients, and chronic immune stimulation, such as in patients with chronic viral infections or autoimmune diseases. There is also a strong association with certain genetic abnormalities, particularly isochromosome 7q, which is found in the majority of cases. Additionally, some studies have suggested a potential link between the use of immunomodulatory drugs, such as those used to treat inflammatory bowel disease, and the development of HSTCL, although this association remains controversial.
Who gets Hepatosplenic T-Cell Lymphoma?
According to the National Cancer Institute, Hepatosplenic T-Cell Lymphoma makes up <1% of all new non-Hodgkins lymphoma diagnoses.