Hepatosplenic T-Cell Lymphoma (HSTCL) is a rare type of T-cell (a type of white blood cell or lymphocyte) lymphoma. It is aggressive, fast-growing cancer affecting the liver, spleen, and sometimes the bone marrow. In most cases, HSTCL is usually fatal.
Signs of HSTCL
One of the most common signs of HSTCL is an enlarged liver or spleen, with symptoms including abdominal pain or fullness. The disease progresses quickly spreading to the spleen, liver, and usually the bone marrow. Symptoms include fever, fatigue, jaundice, and infections. Tests often reveal abnormal liver function and reduced peripheral blood cells.
Abbott reports that most HSTCL cases occurred in patients with Crohn’s or ulcerative colitis (inflammation of the digestive system). In addition, most of these people had also been treated with immunosuppresants (azathiprine or 6-mercaptopurine) before or at diagnosis of HSTCL. However, Abbott reports that it is not known whether HSTCL is related to use of Humira (or other TNF blocker) or a TNF blocker in combination with immunosuppressants.
A disproportionate risk
What is known is that HSTCL is a rare form of cancer and a disproportionate number of Humira users have suffered from this dangerous and aggressive form of cancer. Even more disheartening is the fact that cases of HSTCL linked to Humira are more often seen in adolescent and young adult males.
- In June 2008, the FDA first communicated about the increased risk of cancer associated with use of TNF blockers. This release was in response to about thirty reports of cancer in children and young adults that the FDA was investigating. The cancer reports were submitted to the FDA’s Adverse Event Reporting System over a ten-year period beginning in 1998 after the approval of the first TNF blocker.
- Then, in August 2009, the FDA required cancer warnings on the labels of TNF blockers. The FDA also released a follow-up to the June 2008 communication about the safety of TNF blockers. Following its analysis of TNF blockers, the FDA concluded that there was an increased risk of lymphoma and cancer associated with the drugs in both children and adolescents.
- On April 14, 2011, the U.S. Food and Drug Administration (FDA) released a Communication because of continuing reports of cases of HTCL in people taking TNF blockers (including Humira). Most reported cases of HSTCL occurred in patients taking a combination of TNF blockers and immunosupressants, but cases have also been reporting in people taking only immunosuppressants. The FDA updated the product label warning for Humira to include a warning about HSTCL. However, this warning came too late for some Humira users.
Unfortunately, because the aggressive nature of HSTCL, most cases are fatal. However, there are treatment options, but the aggressive cancer requires aggressive treatment. Treatment options include chemotherapy, removal of diseased spleen (splenectomy), bone-marrow transplant, The Cancer Survivors Network, has personal stories and information from patients and family members about their treatment of HSTCL. Much of the treatment involves supportive care to manage infections. Unfortunately, even the most aggressive of treatment options cannot cure HSTCL.