Introduction: Hepatosplenic T-cell lymphoma (HSTCL) is a rare form of non-Hodgkin lymphoma with unique features including presentation primarily in young men, lymphomatous infiltration of the liver and spleen, frequent bone marrow involvement, B symptoms, infrequent lymphadenopathy and poor prognosis. First described by Farcet and Gaulard1, there are two larger published series in whom only 6/66 of patients (pts) were alive at the time of the reports. 2,3 4/6 surviving pts in these reports had undergone high dose therapy and autologous or allogeneic stem cell transplantation (HDT-SCT). There are no prospective studies of treatment of HSTCL but a recent review of published case reports of HSTCL treated with allogeneic SCT suggests a better outcome for that approach.4

Methods: We reviewed our T-cell lymphoma and bone marrow transplantation databases to examine our results in pts with HSTCL. We identified 9 consecutive pts with this diagnosis. This report summarizes our single center experience.

Results: All pts were male with a median age of 37y (12–59). All pts had stage IV disease with hepatomegaly and/or splenomegaly. 5/9 had documented bone marrow involvement, 7 had elevated LDH, and all 9 had B symptoms. Thrombocytopenia was present at diagnosis in 5 pts, anemia in 4 pts, and leukopenia in 4 pts. Transaminases and/or alkaline phosphatase were elevated in 6 pts. 4/9 had previous autoimmune disease: 2 with ulcerative colitis and 2 with rheumatoid arthritis. Responses to induction regimens were: CHOP (PR-2, POD-1) ICE/IVAC (CR-2, PR-2), pentostatin/2-CDA (POD-2). 2/4 pt achieved a CR to ICE as second line therapy. 8/9 pts achieved at least a PR and proceeded to HDT-SCT. 6 pts received an allogeneic SCT (one after relapse from autologous SCT), and 3 pts an autologous SCT. At the time of this report, 4/9 patients are alive in remission, 20–158 mos from diagnosis; the 4 surviving patients all underwent HDT/SCT. Following autologous-SCT 2/3 pts relapsed at 5 and 35 mos. Following Allogeneic-SCT 2/6 pts relapsed at 3 and 6 mos, 1 of whom was effectively treated with donor lymphocytes and remains in remission at 20 mos. 2/6 pts undergoing allo-SCT died of treatment related toxicities without documented recurrent disease. Complete information to determine the age-adjusted international prognostic index (aaIPI) was available for 8/9 pts; the aaIPI appeared to correlate with outcome: 4/5 pts with an aaIPI of low intermediate to high intermediate risk (1–2 factors) were alive compared to 0/3 aaIPI high risk disease (3 factors). The prognostic index for PTCL (PIT) consisting of age, performance status, LDH, and bone marrow involvement was also assessed. All 8 pts had at least one risk factor; 4/6 pts with a PIT of 1–2 were alive vs 0/2 pts for PIT of ≥3. Four pts received ICE or IVAC as their initial therapy and 3/4 were alive compared to only 1/5 for those who received other initial regimens.

Conclusions: In this single institution experience, use of non-CHOP induction chemotherapy regimens such as ICE or IVAC and early use of HDT-SCT consolidation appear to improve the outcome for pts with HSTCL compared to reported results with CHOP or CHOP-like regimens.

Farcet et al Blood
Weidmann et al Leukemia
Belhadj et al Blood
Konuma et al, Leukemia and Lymphoma

Author notes

Disclosure: No relevant conflicts of interest to declare.