International Journal of Hematology

, Volume 96, Issue 1, pp 136–140

Acute myeloid leukemia with mediastinal myeloid sarcoma refractory to acute myeloid leukemia therapy but responsive to l-asparaginase

  • Hiroyoshi Takahashi
  • Katsuyoshi Koh
  • Motohiro Kato
  • Hiroshi Kishimoto
  • Eiji Oguma
  • Ryoji Hanada
Case Report

DOI: 10.1007/s12185-012-1111-0

Cite this article as:
Takahashi, H., Koh, K., Kato, M. et al. Int J Hematol (2012) 96: 136. doi:10.1007/s12185-012-1111-0

Abstract

We report the case of a 14-year-old female with acute myeloid leukemia (AML) and myeloid sarcomas (MS) in the anterior mediastinum and around numerous bones. Laboratory tests showed a white blood cell count of 4.0 × 109/l with 7.0 % blasts. Computed tomography revealed a mediastinal mass and pleural effusion; pleural effusion cytology was negative for malignant cells. In addition, disseminated intravascular coagulation (DIC) was present. Following DIC therapy, thoracoscopic and bone marrow biopsies were performed. Immunostaining and surface marker analysis revealed that the blast cells were positive for cytoplasmic myeloperoxidase, CD4, CD7, CD33, CD44, CD117, and HLA-DR, but negative for CD34 and CD56. Karyotype was normal. MS associated with AML was diagnosed. Multidrug chemotherapy for AML was completely ineffective, and MS continued to progress. Immunohistochemistry revealed that the blasts were negative for asparagine synthetase (AS); therefore, chemotherapy including l-asparaginase was initiated. After the first administration of l-asparaginase, the patient’s condition improved; however, she subsequently developed tumor lysis syndrome and sepsis, which eventually led to death. Aggressive MS in childhood is rare and refractory to existing AML chemotherapy. Chemotherapy including l-asparaginase may prove to be effective in such cases, especially those in which blast cells show negative AS expression.

Keywords

Acute myeloid leukemia Myeloid sarcoma Mediastinal tumor l-asparaginase CD44 

Copyright information

© The Japanese Society of Hematology 2012

Authors and Affiliations

  • Hiroyoshi Takahashi
    • 1
  • Katsuyoshi Koh
    • 1
  • Motohiro Kato
    • 1
  • Hiroshi Kishimoto
    • 2
  • Eiji Oguma
    • 3
  • Ryoji Hanada
    • 1
  1. 1.Department of Hematology/OncologySaitama Children’s Medical CenterSaitamaJapan
  2. 2.Department of PathologySaitama Children’s Medical CenterSaitamaJapan
  3. 3.Department of RadiologySaitama Children’s Medical CenterSaitamaJapan

Personalised recommendations