Long-Term Survival after T-cell Lymphoblastic Lymphoma Treated with One Cycle of Hyper-CVAD Regimen

Abstract

T-lymphoblastic lymphoma (T-LBL) is a rare form of aggressive non-Hodgkin's lymphoma. The standard approach for management of T-LBL involves intensive multiagent chemotherapy regimens for induction and consolidation phases with central nervous system prophylaxis and a maintenance phase lasting 12-18 months. We report on a case of long-term survival after one cycle of hyperfractionated cyclophosphamide, vincristine, doxorubicin, and dexamethasone (hyper-CVAD) and high-dose methotrexate. A 30-year-old woman diagnosed with T-LBL with a large mediastinal mass underwent one cycle of hyper-CVAD. Four days after the start of treatment, the mediastinal mass was markedly reduced. Treatment continued with one cycle of consolidation chemotherapy, comprising high-dose methotrexate and high-dose cytarabine. The patient then refused all further chemotherapeutic treatment. Seven years have passed without relapse.

Keywords: CVAD protocol; Precursor T-cell lymphoblastic leukemia-lymphoma; Remission induction.

Fig. 1.

Computed tomography (CT) at initial diagnosis. (A) Chest CT scan showed a large mediastinal mass, pleural effusion. (B) Abdominal CT scan showed left para-aortic lymphadenopathy.

Fig. 2.

Microscopic findings of the precursor T-cell lymphoblastic lymphoma from the axillary lymph node. (A) Most tumor cells show immature nuclei with fine chromatin, convoluted nuclear contours, and frequent mitotic figures (H&E staining, ×1,000). On immunohistochemical staining, tumor cells were positive for CD3 (B) CD5 (C), and CD99 (D) (B-D, ×400).

Fig. 3.

Comparison of chest X-ray. (A) Before treatment of hyperfractionated cyclophosphamide, vincristine, doxorubicin, and dexamethasone (hyper-CVAD), on April 3, 2006. (B) Chest X-ray showed that the mediastinal mass had decreased after performance of hyper-CAVD, on April 7, 2006.

Fig. 4.

Comparison of position emission tomography-computed tomography (PET-CT), before and after treatment. (A) In March 2006, PET-CT showed the hypermetabolic mediastinal mass with multiple lymphadenopathy and pleural invasion. (B) In November 2006, after chemotherapy, PET-CT showed no hypermetabolic lesion.