Hepatosplenic T Cell Lymphoma

Disease Summary

Hepatosplenic 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 Features

The 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.

Disclaimer: This site is purely to share information. While we make every attempt to ensure that the information displayed here is current, we cannot guarantee it, nor is it meant to guide treatment.

Treatment

Chemotherapy 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 Research

We 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.

Discussion Forum

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.

Posted on May 12, 2017, 10:05 PM by Mary, Family or Friend,
My son died of the disease when he was just 18 years old. Hepatosplenic is a very deadly disease. My son was on a ventilator for almost two months, he suffered so much. My husband and I never left the hospital. Each day we hoped and prayed he would get better. Our prayers were never answered. In the years since my son’s passing I have done a lot of research to see if I could find a cause. I have emailed countless experts on the subject and they have no answers. I’ve read articles that explain how low levels of Vitamin D can cause Leukemia and colon-rectal cancers. My son had a lymphoma that acted like leukemia. No solid tumor, it was a blood cancer. My husband was diagnosed with rectal cancer within two years after my son died. Soon after reading the article, my family had our Vitamin D levels checked. The results were very alarming. My husband was around 30, my daughter was 21, and I was around 29. Many experts believe the optimal level is 50 to 100. Although my son (Daniel) was never checked for his level of vitamin D, I do know my daughter was a perfect match for his bone marrow transplant. After finding out our levels of Vitamin D we all take 4000 units daily. Even with taking 4000 units my level is 50. Still on the low side of average. One of the ways to get natural vitamin D is sunlight. Yet Dermatologist say, “you can get skin cancer from the sun, make sure you where sunscreen”. We are in a catch 22. We need vitamin D, but don’t go outside without your sunscreen on. Let’s face it; living in the Midwest we don’t get a lot of sun. Another very important issue is the drugs that are used to suppress the immune system for Rheumatoid Arthritis. Humira is one such drug. One of the side effects is the deadly Hepatosplenic T Cell Lymphoma. If you research this Lymphoma you will find people that take this drug, although rare, are getting this disease. I have to wonder if the doctors are prescribing vitamin D along with taking this drug. I keep seeing the ad for Humira and other similar drugs that suppress your immune system, it terrifies me. Mary