Hepatosplenic T-Cell Lymphoma (HSTCL)

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. No specific cause of HSTCL has been identified so far. However, in some cases, long immunosuppression has been implicated. 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.

Once the diagnosis of HSTCL is established or suspected, patients benefit from a referral to specialized academic centers that have experience in managing patients with rare lymphomas. Based on current knowledge of this cancer, most of the patients should be considered for intensive treatment protocols and bone marrow transplantation. Specific treatment plans that suit a particular patient should be discussed with a treating oncologist.

Given the extreme rarity of HSTCL and the lack of established approaches, we encourage patients with this cancer to participate in clinical trials (if available) and specialized registries for T-cell lymphomas — both to gain access to new treatments and to advance knowledge regarding the best way of curing this lymphoma.