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Natural killer/T-cell lymphomas in pediatric and adolescent patients.

Abstract Natural killer/T-cell (NK/T-cell) lymphomas are rare in children and adolescents and consist predominantly of nasal-type extranodal NK/T-cell lymphomas. More than half of pediatric/adolescent patients with NK/T-cell lymphomas present with localized nasal/sinus involvement, but the disease may involve many organs. NK/T-cell lymphoma cells are cytotoxic and associated with necrosis and angioinvasion; they express CD56, CD2, cytoplasmic CD3 epsilon, and to a variable degree CD30. The cells contain Epstein-Barr virus (EBV)-encoded RNA. Loss of chromosome 6q is frequent, and multiple other genetic changes may occur. The Janus kinase/signal transducers and activators of transcription (JAK/STAT) and other pathways are activated in NK/T-cell lymphoma. Adults with stage I/II disease receive radiation with or without chemotherapy, whereas adults with advanced disease receive multiagent chemotherapy, including asparaginase and drugs not affected by P-glycoprotein-mediated resistance. Outcomes data for pediatric patients come from retrospective reviews and retrospective case series. The overall survival of pediatric patients is 77% for those with stage I/II disease and 36% to 59% for those with advanced disease. Bone marrow transplant (BMT) is used in children, but with little evidence regarding the indications and rationale for type of transplant. BMT achieves better outcomes for adult patients in remission, but with high levels of morbidity and mortality. Improved understanding of the biology of this disease will allow the development of targeted approaches, including JAK/STAT inhibitors, checkpoint inhibitors, anti-CD30 agents, epigenetic modifiers, and reduced-intensity conditioning for BMT, to improve outcomes in pediatric patients.
PMID
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Authors

Mayor MeshTerms
Keywords
Journal Title clinical advances in hematology & oncology : h&o
Publication Year Start




PMID- 28398275
OWN - NLM
STAT- MEDLINE
DA  - 20170411
DCOM- 20170420
LR  - 20170420
IS  - 1543-0790 (Print)
IS  - 1543-0790 (Linking)
VI  - 15
IP  - 3
DP  - 2017 Mar
TI  - Natural killer/T-cell lymphomas in pediatric and adolescent patients.
PG  - 200-209
AB  - Natural killer/T-cell (NK/T-cell) lymphomas are rare in children and adolescents 
      and consist predominantly of nasal-type extranodal NK/T-cell lymphomas. More than
      half of pediatric/adolescent patients with NK/T-cell lymphomas present with
      localized nasal/sinus involvement, but the disease may involve many organs.
      NK/T-cell lymphoma cells are cytotoxic and associated with necrosis and
      angioinvasion; they express CD56, CD2, cytoplasmic CD3 epsilon, and to a variable
      degree CD30. The cells contain Epstein-Barr virus (EBV)-encoded RNA. Loss of
      chromosome 6q is frequent, and multiple other genetic changes may occur. The
      Janus kinase/signal transducers and activators of transcription (JAK/STAT) and
      other pathways are activated in NK/T-cell lymphoma. Adults with stage I/II
      disease receive radiation with or without chemotherapy, whereas adults with
      advanced disease receive multiagent chemotherapy, including asparaginase and
      drugs not affected by P-glycoprotein-mediated resistance. Outcomes data for
      pediatric patients come from retrospective reviews and retrospective case series.
      The overall survival of pediatric patients is 77% for those with stage I/II
      disease and 36% to 59% for those with advanced disease. Bone marrow transplant
      (BMT) is used in children, but with little evidence regarding the indications and
      rationale for type of transplant. BMT achieves better outcomes for adult patients
      in remission, but with high levels of morbidity and mortality. Improved
      understanding of the biology of this disease will allow the development of
      targeted approaches, including JAK/STAT inhibitors, checkpoint inhibitors,
      anti-CD30 agents, epigenetic modifiers, and reduced-intensity conditioning for
      BMT, to improve outcomes in pediatric patients.
FAU - Termuhlen, Amanda M
AU  - Termuhlen AM
AD  - Keck School of Medicine, University of Southern California in Los Angeles,
      Children's Center for Cancer and Blood Diseases, Children's Hospital Los Angeles,
      Los Angeles, California.
LA  - eng
PT  - Journal Article
PT  - Review
PL  - United States
TA  - Clin Adv Hematol Oncol
JT  - Clinical advances in hematology & oncology : H&O
JID - 101167661
SB  - IM
MH  - Adolescent
MH  - Age Factors
MH  - Child
MH  - Child, Preschool
MH  - Combined Modality Therapy
MH  - Genetic Predisposition to Disease
MH  - Genetic Variation
MH  - Humans
MH  - Infant
MH  - Infant, Newborn
MH  - Lymphoma, T-Cell/*diagnosis/epidemiology/etiology/*therapy
MH  - Natural Killer T-Cells/*pathology
MH  - Prognosis
MH  - Signal Transduction
MH  - Treatment Outcome
EDAT- 2017/04/12 06:00
MHDA- 2017/04/21 06:00
CRDT- 2017/04/12 06:00
PST - ppublish
SO  - Clin Adv Hematol Oncol. 2017 Mar;15(3):200-209.

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