The survival rate of Adult T-cell leukemia-lymphoma (ATL) patients, especially those who develop the acute leukemic or lymphomas forms, is very poor, and such clonal malignant CD4 expansion remains one of the most severe lymphoproliferations. We conducted a retrospective analysis of 31 consecutive patients diagnosed with HTLV-1 adult T-cell lymphoma-leukemia (ATL), treated at a single institution between 1995–2004. We used analysis of variance examining the mean survival of the patients. The patients were stratified according to the treatment conventional CHOP chemotherapy versus non-CHOP chemotherapy (CVP, no therapy or AZT and interferon). Twenty-two patients have received CHOP chemotherapy and 9 patients have not received CHOP chemotherapy. The mean survival for the entire group of patients was 22.7 weeks. The median survival was 16 weeks (range from 1–74 weeks). Most of the patients were at high IPI risk. (22 patients IPI=4, 8 patients IPI 5 and one patient IPI 3). If the patients presented without hypercalcemia their mean survival was significantly higher than the patients with hypercalcemia, 22.7 weeks versus 16 weeks p<0.05.

The treatment with CHOP chemotherapy significantly improved survival mean 29.3+4.6 weeks versus 8.7 +2.5 weeks, p=0.01. The treatment with CHOP consistently improved survival in patients without hypercalcemia and in those with hypercalcemia 38.8+ 5.4 weeks versus 5.5+ 12.1 and in those with hypercalcemia 20.7+5.2 weeks versus 9.5+6 weeks. CHOP chemotherapy helped both groups of patients those who presented with hypercalcemia and those without hypercalcemia although there is a suggestion that it is less effective in patients with hypercalcemia. In this small retrospective study, no significant association was found between survival and presence or absence of pleural effusion, ascites, extensive liver or skin involvement. Hypercalcemia should be considered a poor prognostic factor in ATL patients.

Prospective multi-institution clinical trials are crucial to determine the most effective treatment regimens that will continue to improve survival rates in this aggressive disease.

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