Treatment of a Case of T-Lymphoblastic Lymphoma with Unrelated Cord Blood Stem Cell Transplantation

Jiang, Z.; Jiang, S.; Jiang, Q.; He, C.; Luo, Y.

One pediatric T-lymphoblastic lymphoma (T-LBL) has been transplanted with unrelated cord blood progenitor cells and has been disease-free survival for two years. She is an 8-year-pupil. She had pain in her right eye with a red mass in her eyelid on May 1998. A mass increasing in diameter and was of 20x20 mm appeared in her right parotid gland on Jun 1999. Pathological test showed T-LBL. There were 6.0% of lymphoblastic cells in her bone marrow and the blasts in her center nervous system fluid after the two courses of NHL-type chemotherapy. She had CR after receiving ALL-type regimen (DNR, VCR, L-ASP, and Pred). Then she received 4 times of intrathecal injection (DXM, MTX, and Ara-C) and intensive chemotherapy with DXM and CY, admycin, Vindesine and Cytarabine. He then received unrelated, ABO major and minor incompatibility, HLA partially matched cord blood cell transplantation. The donor was from Shanghai Blood Bank. The ABO blood group of donor is O .The receipt's was A. HLA type of the donor and receipt is HLA-A2/-, HLA-B 46/60, HLA-DR 9/12, and HLA-A 2/33, HLA-B 46/60, HLA-DR 9/12, respectively. The condition regimen consisted of total body irradiation (400cGy, -6d, -5d) and CY (60mg/kg, -4d, -3d), VP-16 (18mg/kg, -4dapprx -2d). The cord blood cells transfused on July 9, 2000. The dose of mononuclear cells was 1.538x109 included CD34 positive cells was 0.833%. The dose of CFU-GM colony was >100/1.0x105, BFU-E was 15/1.0x105. The regimen of prophylactic GVHD consisted with the combination of ATG (2.5 mg/kg, -4dapprx-2d), CsA (2mg/kg, -1apprx) and MTX (10mg, +1, +3, +6, +11d). His WBC decreased to 0 at +3d and had remained 0 for 13d after CBT. The red rash skin and white piebald skin appeared in her neck and around her ear at +21d and had disappeared after treating with methylprednisolone (60mg/d daily) for seven days. She had fungi and several bacterial infections in oral cavity and gastrointestinal tract. Her hemapoietic recovery was at +25d. The HLA phenotype changed to O type at +45d. She has moved out from the air laminar flow room at +53d. Her blood group changed from O to A at +57d. The gene map test showed donor's picture. She has had disease-free survival for two years. She is regular normal live and study under our following-up now. She has been to his school for 1 and half year. T-LBL cells is same as acute T-ALL cells to tend to infiltrate some organs such as bone marrow, CNS, spleen that results in relapse. This disease also can be called as T-lymphoblastic lymphoma/leukemia. Some pathologist thought T-LBL and T-ALL were a disease but two names. The patient with T-LBL had lymphoblastic cells infiltration in bone marrow and CNS. Clinicians think the disease should be treated ALL-type chemotherapy. BFM Group reported LBL patients have 80%-90% long-term disease-free survival. Some refractory patients need progenitor cells transplantation. Pre-transplantation therapy is very important for the waiting patients. So they should give ALL-type regimen as pre-transplantation therapy. The patient underwent CBT showed successful graft. His blood group, HLA-type and gene map has changed to donor's picture. Although she has disease-free survival for two years, she still need under our follow-up.