A Case of Hepatosplenic Gamma Delta T Cell Lymphoma With Concomitant Bone Marrow Aplasia Following Azathioprine Therapy, Treatment Course and Review of the Literature

Background

Hepatosplenic T-cell lymphoma (HSTCL) is a rare and aggressive subtype of peripheral T-cell lymphoma with a poor prognosis, primarily affecting young adult males, many with a background of immunosuppression or autoimmune disease.

Case

We present the case of a 27-year-old male previously treated with azathioprine who developed pancytopenia. Bone marrow biopsy revealed severe aplasia with partial infiltration by gamma-delta T-lymphocytes (Tγδ). A definitive diagnosis of HSTCL was established through liver biopsy. The patient received induction chemotherapy with the Ifosfamide, Carboplatin, Etoposide (ICE regimen) but remained pancytopenic, prompting allogeneic stem cell transplantation. Sixty months post-treatment, he remains in complete remission.

Conclusion

To our knowledge, this is the first reported case of HSTCL presenting concurrently with aplastic anemia in the context of prior azathioprine exposure. The underlying pathophysiology is likely multifactorial, involving both immune-mediated suppression and drug-induced toxicity. Clinicians should maintain a high index of suspicion for lymphoma in patients developing unexplained pancytopenia while on azathioprine therapy.