Pediatric Leukemia of Natural Killer Cells: Diagnosis and Multi-Agent Chemotherapy

  • Ashwin Patel
  • Somsuvra Ghatak
Part of the Pediatric Cancer book series (PECA, volume 4)


Natural killer (NK) cell-derived neoplasms are a group of uncommon, heterogeneous and highly aggressive malignancies mainly seen in Asian and South American populations. The involvement of children is less common compared to adults and hence exclusive pediatric data is lacking and many management decisions are based on adult data. They are classified by the World Health Organization (WHO) into extranodal NK/T-cell lymphoma (nasal and extranasal) (ENKL) and aggressive NK cell leukemia (ANKL). NK cell lymphoblastic lymphoma and chronic lymphoproliferative disorder of NK cell are included in provisional category. While myeloid/NK cell leukemia is considered as a type of AML and Hematodermic NK cell neoplasm is considered dendritic cell neoplasm. NK cell neoplasm is characterized by polymorphic neoplastic infiltrate, angioinvasion and/or angiodestruction, cytoplasmic azurophilic granules, CD2+/surface CD3/cytoplasmic CD3ε+/CD56+ phenotype and Epstein-Barr virus (EBV) infection. Cytogenetic study shows recurrent aberrations of 6q, 11q, 13q, and 17p. T cell receptor (TCR) genes are always in germline configuration. Mutations of p53, C-KIT, FAs gene and B-catenin are frequently seen. NK-cell neoplasms are often resistant to conventional combination chemotherapy due to p-glycoprotein expression and associated multidrug resistance. L-asparaginase based chemo­therapy gives better outcome. The prognoses of both localized and advanced stages of NK-cell malignancies are worse than majority of other lymphoid malignancies. Stem cell trans-plantation is recommended for the advanced/ relapsed disease. Studies are currently underway to assess the safety, efficacy and outcomes of chemotherapy, molecular pathway inhibitors and monoclonal antibodies.


Natural Killer Cell Hematopoietic Stem Cell Transplantation Allogeneic Hematopoietic Stem Cell Transplantation Autologous Hematopoietic Stem Cell Transplantation Large Granular Lymphocyte 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


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Copyright information

© Springer Science+Business Media Dordrecht 2013

Authors and Affiliations

  1. 1.The Gujarat Research and Medical InstituteAhmedabadIndia
  2. 2.Department of Pharmacology, Institute of PharmacyNirma UniversityAhmedabadIndia

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