Treatment of a Child With Myeloid/NK Cell Precursor Acute Leukemia With L-Asparaginase and Unrelated Cord Blood Transplantation
- 119 Downloads
A 2-year-old Japanese boy who presented with multiple cervical, axillary, and inguinal lymphadenopathy was diagnosed by immunocytochemical analysis as having myeloid/natural killer (NK) cell precursor acute leukemia. Leukemic blasts in the bone marrow were positive for CD56 (NK marker), CD7 (T-cell marker), CD33 (myeloid marker), CD34, and HLA-DR. Tumor cells in a lymph node were also positive for CD2, cytoplasmic CD3 (T-cell marker), CD7, CD33, CD34, and CD56, but negative for peroxidase staining and other T-cell, NK, and myeloid markers. Southern blot analysis showed no rearrangement bands for T-cell receptor δ and immunoglobulin heavy chain. Chromosomal analysis revealed 46,XY,inv(7)(p21q21). Neither chemotherapy for acute lymphoblastic leukemia nor that for acute myeloid leukemia induced remission in this patient. How-ever, complete remission was achieved by the administration of L-asparaginase (6000 U/m2 for 5 days). Because the disease was considered refractory to standard chemotherapy, cord blood transplantation was performed from an HLA 1-locus mis-matched unrelated donor. The conditioning regimen consisted of total body irradiation, cytarabine, and cyclophosphamide, and cyclosporine and short-term methotrexate were employed for graft-versus-host disease (GVHD) prophylaxis. Hematological reconstitution was rapid, and only grade I acute GVHD was observed. The patient has been in remission for more than 24 months after transplantation. Our findings indicate that combination therapy with L-asparaginase and allogeneic stem cell transplantation may be useful for the treatment of myeloid/NK cell precursor acute leukemia.
Key wordsChildhood leukemia Myeloid/NK cell precursor Chromosomal abnormality Cord blood transplantation L-asparaginase
Unable to display preview. Download preview PDF.
- 1.Suzuki R, Yamamoto K, Seto M, et al. CD7 and CD56 myeloid/natural killer cell precursor acute leukemia: a distinct hematolymphoid disease entity.Blood. 1997;90:2417–2428.Google Scholar
- 3.Scott AA, Head DR, Kopeckey KJ, et al. HLA-DR-, CD33+, CD56+, CD16- myeloid/natural killer cell acute leukemia: a previously unrecognized form of acute leukemia potentially misdiagnosed as French-American-British acute myeloid leukemia-M3.Blood. 1994;84:244–255.Google Scholar
- 9.Kuetzberg J, Waldmann TA, Davey MP, et al. CD7+, CD4-, CD8-, acute leukemia: a syndrome of malignant pulripotent lymphohematopoietic cells.Blood. 1989;73:381–390.Google Scholar
- 12.Kitoh E, Nishimura S, Shirahase H, et al. Immunocytochemical analysis of asparagine synthetase protein of leukemia cells: indication for L-asparaginase treatment [abstract].Blood. 1996;88(suppl 1):86a.Google Scholar
- 13.Kitoh T, Sawada M, Shirahase H, et al. Asparagine synthetase protein expression in leukemia cells: application of L-asparaginase treatment for leukemia [abstract].Blood. 1998;92(suppl 1):400a.Google Scholar
- 14.Rocha V, Cornish J, Sievers E, et al. Comparison of the outcome of unrelated bone marrow (BM) or cord blood (CB) transplantation in children with acute leukemia (AL) [abstract].Blood. 1999;94(suppl 1):712a.Google Scholar
- 17.Yoshino H, Tanaka R, Yoshimaru T, et al. Allogeneic bone marrow transplantation from the HLA one-locus mismatched father for a child of myeloid/NK cell precursor acute leukemia with late relapse [in Japanese; abstract].The 42nd Annual Meeting of Japanese Society of Pediatric Hematology. 2000;14:85.Google Scholar