A Case of Hepatosplenic Gamma-delta T Cell Lymphoma with Intravascular Lymphoma Like Features in a Young Male With Autoimmune Hepatitis

Abstract

Hepatosplenic gamma delta T-cell lymphoma (HSGDTL) is a rare and aggressive hematologic malignancy that has been associated with long term use of immunosuppressive agents. Here we report a 22-year-old male on azathioprine and prednisone for autoimmune hepatitis who presented to hospital with abdominal pain, night sweats and 20lbs of weight loss. On exam he had hepatomegaly and splenomegaly but no lymphadenopathy. His initial lab tests were WBC 5.39 x 109/L, Hb 70 g/L, platelets 25 x 109/L, total bilirubin 74 μmol/L, AST 151 U/L, ALT 55 U/L and LDH 5932 U/L. He had marked hepatosplenomegaly without lymphadenopathy on abdominal CT scan. Bone marrow and liver biopsies confirmed the presence of HSGDTL. Unfortunately he did not respond to multiple cycles of chemotherapy and passed away. On autopsy he had multiorgan involvement of an intravascular lymphoma like (IVLL) T-cell population. There are no definitive risk factors for HSGDTL but an association with immunosuppressive agents has been well documented in renal transplant recipients and patients with inflammatory bowel disease (IBD). Specifically, the use of thiopurines for at least two years in young males with IBD has been linked to an increased risk of developing HSGDTL. To our knowledge, however, previous cases of HSGDTL in autoimmune hepatitis patients have been minimally reported in the literature. Unique to our case was the finding of an intravascular lymphoma like component on autopsy that was present in tissue samples from the patient's lungs, kidneys, heart, thyroid and testicles. As far as we know, a widespread multi-organ intravascular lymphoma in a HSGDTL patient is exceedingly rare. In fact we found only one other case in the literature. Therefore, it is difficult to assess the impact of this finding. It is possible the presence of intravascular lymphoma represents a subtype of HSGDTL and may provide insight into the aggressive nature of this disease. We must also consider an association between autoimmune hepatitis and HSGDTL with IVLL component independent of immunosuppressive therapy. However, future research into this little known and seldom reported malignancy will be required to fully appreciate these findings. Overall, we hope our case report will contribute to better understanding the association between T-cell lymphoma and long term use of immunosuppressive agents as well as identify new populations, specifically autoimmune hepatitis patients, who may be at risk of developing this deadly disease.Figure 1