Hepatosplenic T Cell Lymphoma
Disease SummaryHepatosplenic T cell lymphoma (HSTL or HSTCL) is a rare, but lethal cancer. Compared to other T cell lymphomas, HSTL is unusual in that it affects predominantly younger patients (typically patients are in their thirties although the disease can occur at any age). Most HSTL patients succumb to their disease in less than a year.
Genetic FeaturesThe most frequent known genetic abnormalities in HSTL are isochromosome 7q (iso7q) and trisomy 8. The most frequent gene mutations in HSTL occur in STAT5B, SETD2, STAT3, INO80 and PIK3CD.
Welcome to HSTL.Patients
The purpose of this website is to share information about the disease, as well as offer a forum to share information about the disease from the perspectives of patients, their families, their physicians and other caretakers. This website is entirely free.
TreatmentChemotherapy is usually the first-line treatment in HSTL. Unfortunately, relapses are common. Where possible, bone marrow transplantation should be considered. The genetic mutations suggest a potential application of JAK-STAT pathway inhibitors and PI3 kinase inhibitors, which may work well in combination. These therapies have not been tested and their use should be considered very experimental. Reporting experiences with these and other treatments in the forum could be potentially greatly beneficial to other patients.
Learn More/Participate in ResearchWe are continuing to study the risk factors and molecular features of hepatosplenic T cell lymphomas, particularly in patients who have inflammatory bowel disease or other autoimmune diseases. To learn more, please register here and we will contact you.
Feel free to share any aspect of your experience from how the disease was diagnosed to treatment and response. Please identify your role clearly (patient, family or friend, or professional caregiver). You do not need to provide your real name.