The Basics About Hepatosplenic T-cell Lyphoma

• The main risk factors associated with HSTCL include continued use of immunosuppression systemic therapy for inflammatory bowel disease such as Crohn's Disease.

• Hepatosplenic T-cell Lymphoma has an overall survival rate of roughly 7%.  The reported median survival of patients with HSTCL is 6 to 11 months following diagnosis. 

• Hepatosplenic T-Cell Lymphoma (HSTCL) is a very rare lymphoma. This illness has only rarely been reported in the scientific literature, and so its true incidence is unknown

• The incidence of Hepatosplenic T-Cell Lymphoma (HSTCL) has at least doubled in the past decade. The overall survival rate remains poor and disturbingly has not improved over time. 

• Hepatosplenic T-cell Lymphoma (HSTCL) is a rare form of peripheral T-cell lymphoma. It is associated with an aggressive clinical course, a poor response to conventional treatment, and an exceedingly high mortality rate. 

• Once the diagnosis of Hepatosplenic T-cell Lymphoma  is confirmed therapy should be initiated promptly as the disease can progress quite rapidly. 

• No standard therapy exists for HSTCL, owing to the rarity of this disease, however, chemotherapy regimens, stem cell transplant and participation in clinical trials may be among the options considered.

Source: https://www.tcllfoundation.org/

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.