Importance of early splenectomy in patients with hepatosplenic T-cell lymphoma and severe thrombocytopenia.

Objective: T-cell lymphomas (TCLs) classically have a poorer response to therapy when compared with B-cell lymphomas and account for only 10-15% of all lymphoid malignancies. Hepatosplenic TCLs are a rare subset of this group that usually present with hepatosplenomegaly, B-symptoms, and only rarely with lymphadenopathy.

Background: This disease process, also known as gamma/delta (gamma/delta) TCL because of the expression of the T-cell receptor gamma/delta chain, tends to present in young male patients. Hepatosplenic TCLs have recently gained notoriety because of the realization that patients with long-term treatment of some immunomodulators can develop this potentially fatal disease. These facts are exacerbated by the fact that patients with this disease rarely enjoy remissions of more than brief duration with common chemotherapeutic agents or bone marrow transplants. A novel agent, pralatrexate, has recently been found to have a dramatic activity in this patient population with refractory/relapsed disease. Unfortunately, patients with hepatosplenic TCL often present with thrombocytopenia and this new agent is contraindicated in these patients because of the potential exacerbation of thrombocytopenia with this agent.

Methods: Because of this we attempted a laparoscopic-assisted splenectomy in one patient with grade 4 thrombocytopenia.

Results: Postoperatively the patient's thrombocytopenia resolved, permitting him to begin treatment with this potentially life-saving agent.

Conclusion: Due to the lethality of this disease and potential efficacy of new therapies, we believe splenectomy should be considered in patients with hepatosplenic lymphoma in an effort to improve the treatment options and survival of patients with this challenging disease.