Hepatosplenic T-cell lymphoma
|Hepatosplenic T-cell lymphoma|
|Synonyms||hepatosplenic γδ T-cell lymphoma|
|Specialty||Hematology and oncology|
Hepatosplenic T-cell lymphoma is a rare and generally incurable form of lymphoma, except in the case of an Allogenic Stem Cell Transplant. Hepatosplenic T-cell lymphoma is a systemic neoplasm comprising medium-sized cytotoxic T-cells that show a significant sinusoidal infiltration in the liver, spleen, and bone marrow.
Signs and symptoms
Sites of involvement
The cell of origin for this disease is an immature cytotoxic T-cell clonally expressing the γδ T-cell receptor. This disease is seen more often in immunosuppressed solid organ transplant recipients, an association that has led to the hypothesis that long-term immune stimulation in the setting of immunosuppression is the causative agent.
Cases of hepatosplenic T-cell lymphoma (HSTCL) have been reported in patients treated with the immunosuppressants azathioprine, infliximab and adalimumab. The majority occurred in patients with inflammatory bowel disease. Adolescents and young adult males were the major cohort of cases. They presented with a very aggressive disease course and, with one exception, died of the lymphoma. The FDA has required changes to the labeling to inform users and clinicians of the issue.
Spleen and liver
While the bone marrow is commonly involved, the detection of the neoplastic infiltrate may be difficult due to diffuse, interstitial pattern. Immunohistochemistry can aid in the detection of this lymphoma.
|Positive||CD3, TCRδ1, TIA-1|
|Negative||CD4, CD5, CD8|
Clonal rearrangement of the γ gene of the T-cell receptor is the hallmark of this disease. A few cases have shown rearrangement of the T-cell receptor β gene. Isochromosome 7q has been observed in all cases described so far, sometimes in conjunction with other chromosomal abnormalities such as trisomy 8.
CHOP frequently induces remission initially, but has proven weak compared to treatments that integrate cytarabine with Hyper-CVAD being particularly effective, with chemo therapy only treatment most patients relapse and die within two years. Doxirubicin only treatment can make this disease much worse.Autologous bone marrow transplantation is currently being investigated in the treatment of hepatosplenic lymphoma. Allogeneic bone marrow transplant has been proven to attain remission for over five years and possibly cure hepatosplenic lymphoma with a number of patients surviving if they can make it to Allogenic transplant. 
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