Tommy lived with Crohn's Disease since age 9, taking 6MP, the likely cause of his case of HSTCL, to treat his condition. At age 20, Tommy's semi-annual blood work returned the first signs of HSTCL.
His white blood cell counts had fallen off. This condition may have pre-existed for up to 6 months, since his prior normal blood tests were taken.
It took doctors another 8 months to finally diagnose Tommy with HSTCL while they performed various tests to determine the cause of his symptoms, leaving testing of his bone marrow to a very late date.
By the time Tommy was finally diagnosed, he lost 8-14 months in his opportunity to receive treatment.
Tommy perished less than 8 months after diagnosis.
Hepatosplenic T-cell Lymphoma (HSTCL) is an extremely rare and very unique subtype of peripheral T-cell lymphoma. It usually presents at a younger age and is more common in men than women. Due to its rarity, it is unclear if any particular race is affected more or less often, or if any geographic region of the world has a higher incidence. There is a suggestion that young people who were treated for childhood inflammatory bowel disease (such as ulcerative colitis and Crohn’s disease) might be at risk for developing HSTCL.
Patients with HSTCL frequently have generalized symptoms, such as fatigue, significant night sweats, weight loss and unexplained fevers. Frequently, it is difficult to eat a full meal due to the enlargement of the spleen and pressure on the stomach that it exudes. First symptoms of HSTCL could be frequent nose bleeds or easy bruising from a low platelet count; new or worsened shortness of breath with walking or exercise resulting from low red blood cell count; or frequent infections (such as sinus infections or pneumonias) from low white blood cell counts (all from an enlarged spleen that “traps” a lot of blood cells). Not all of the symptoms can or will be present in the same person affected by HSTCL.
When doctors start evaluating patients with HSTCL, they frequently find very low blood cell counts and abnormalities in liver function on blood tests; enlarged spleen and liver on the CT-scans (of note: lymph nodes or other organs are very rarely enlarged or involved by the lymphoma); presence of abnormal cells in the blood through a specialized test called flow cytometry.
Establishing the diagnosis of HSTCL generally requires biopsies of the bone marrow and liver. Clonal (cancerous) T-cells typically have characteristic appearance under the microscope and on special tests. However, in some cases, making the diagnosis is challenging and requires review by specialized pathologists.
Tommy was more susceptible to HSCTL given that he was taking 6MP, (one of several Crohn's Disease treatments which are known to cause HSCTL) at a young age and for a significant period of time, more than 10 years.
6MP, Humira, Imuran and Remicade are in a family of drugs which have been known to participate in contracting HSTCL. However, given his case, essential testing to determine if Tommy's symptoms were related to HSTCL, were not considered until all other tests had been exhausted. Tommy lost more than 6 months of time during these inconclusive tests, threatening his already small chances of surviving this lymphoma.
We highly recommend that, if you are taking any treatment for Crohn's Disease or Ulcerative Colitis, which "may" cause HSCTL, that you advocate for more aggressive testing, for the purpose of determining if HSTCL is present.
Time to treat HSCTL is your best asset.