International Journal of Hematology

, Volume 75, Issue 2, pp 201–206 | Cite as

Treatment of a Child With Myeloid/NK Cell Precursor Acute Leukemia With L-Asparaginase and Unrelated Cord Blood Transplantation

  • Kayo Tezuka
  • Hideki Nakayama
  • Keiko Honda
  • Junji Suzumiya
  • Kouichi Oshima
  • Toshiyuki Kitoh
  • Eiichi Ishiia
Case Report


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 words

Childhood leukemia Myeloid/NK cell precursor Chromosomal abnormality Cord blood transplantation L-asparaginase 


Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.


  1. 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.PubMedGoogle Scholar
  2. 2.
    Suzuki R, Nakamura S. Malignancies of natural killer (NK) cell precursor: myeloid/NK cell precursor or acute leukemia and blastic NK cell lymphoma/leukemia.Leuk Res. 1999;23:615–624.CrossRefPubMedGoogle Scholar
  3. 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.PubMedGoogle Scholar
  4. 4.
    Nagata T, Higashigawa M, Nagai M, et al. A child case of C34+, CD33-, HLA-DR-, CD7+, CD56+ stem cell leukemia with thymic involvement.Leuk Res. 1996;20:983–985.CrossRefPubMedGoogle Scholar
  5. 5.
    DiGiusppe JA, Louie DC, Williams JE, et al. Blastic natural killer cell leukemia/lymphoma: a clinicopathologic study.Am J Surg Pathol. 1997;21:1223–1230.CrossRefGoogle Scholar
  6. 6.
    Nakamura S, Suchi T, Koshikawa T, et al. Clinicopathologic study of CD56 (NCAM)-positive angiocentric lymphoma occurring in sites other than the upper and lower respiratory tract.Am J Surg Pathol. 1995;19:284–296.CrossRefPubMedGoogle Scholar
  7. 7.
    Sanchez, MJ, Muench MO, Roncarolo MG, et al. Identification of a common T/natural killer cell progenitor in human fetal thymus.J Exp Med. 1994;180:569–576.CrossRefPubMedGoogle Scholar
  8. 8.
    Shibuya A, Nagayoshi K, Nakamura K, et al. Lymphokine requirement for the generation of natural killer cells from CD34+ hematopoietic progenitor cells.Blood. 1995;85:3538–3546.PubMedPubMedCentralGoogle Scholar
  9. 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
  10. 10.
    Inaba T, Shimazaki C, Sumikuma T, et al. Clinicopathological features of myeloid/natural killer (NK) cell precursor acute leukemia.Leuk Res. 2001;25:109–113.CrossRefPubMedGoogle Scholar
  11. 11.
    Obama K, Tara M, Niina K. L-asparaginase induced complete remission in Epstein-Barr virus positive, multidrug resistant, cutaneous T-cell lymphoma.Int J Hematol. 1999;69:260–262.PubMedPubMedCentralGoogle Scholar
  12. 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. 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. 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
  15. 15.
    Locatelli F, Rocha V, Chastang C, et al. Factors associated with outcome after cord blood transplantation in children with acute leukemia.Blood. 1999;93:3662–3671.PubMedGoogle Scholar
  16. 16.
    Mogul MJ. Unrelated cord blood transplantation vs. matched unrelated donor bone marrow transplantation: the risks and benefits of each choice.Bone Marrow Transplant. 2000;25(suppl 2):S58-S60.CrossRefPubMedGoogle Scholar
  17. 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

Copyright information

© The Japanese Society of Hematology 2002

Authors and Affiliations

  • Kayo Tezuka
    • 1
  • Hideki Nakayama
    • 1
  • Keiko Honda
    • 1
  • Junji Suzumiya
    • 2
  • Kouichi Oshima
    • 2
  • Toshiyuki Kitoh
    • 3
  • Eiichi Ishiia
    • 4
  1. 1.Division of PediatricsHamanomachi HospitalFukuoka
  2. 2.First Department of Pathology, School of MedicineFukuoka UniversityFukuoka
  3. 3.Department of PediatricsShiga Medical Center for ChildrenShiga
  4. 4.Department of PediatricsSaga Medical SchoolSagaJapan

Personalised recommendations