Skip to main content

Advertisement

SpringerLink
Log in

Non-anaplastic peripheral T cell lymphoma in children and adolescents—an international review of 143 cases

  • Original Article
  • Published:
Annals of Hematology Aims and scope Submit manuscript

Abstract

Peripheral T cell lymphomas (PTCL) are rare in children and adolescents, and data about outcome and treatment results are scarce. The present study is a joint, international, retrospective analysis of 143 reported cases of non-anaplastic PTCL in patients <19 years of age, with a focus on treatment and outcome features. One hundred forty-three patients, between 0.3 and 18.7 years old, diagnosed between 2000 and 2015 were included in the study. PTCL not otherwise specified was the largest subgroup, followed by extranodal NK/T cell lymphoma, hepatosplenic T cell lymphoma (HS TCL), and subcutaneous panniculitis-like T cell lymphoma (SP TCL). Probability of overall survival (pOS) at 5 years for the whole group was 0.56 ± 0.05, and probability of event-free survival was (pEFS) 0.45 ± 0.05. Patients with SP TCL had a good outcome with 5-year pOS of 0.78 ± 0.1 while patients with HS TCL were reported with 5-year pOS of only 0.13 ± 0.12. Twenty-five percent of the patients were reported to have a pre-existing condition, and this group had a dismal outcome with 5-year pOS of 0.29 ± 0.09. The distribution of non-anaplastic PTCL subtypes in pediatric and adolescent patients differs from what is reported in adult patients. Overall outcome depends on the subtype with some doing better than others. Pre-existing conditions are frequent and associated with poor outcomes. There is a clear need for subtype-based treatment recommendations for children and adolescents with PTCL.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3

Similar content being viewed by others

References

  1. Kontny U, Oschlies I, Woessmann W, Burkhardt B, Lisfeld J, Salzburg J, Janda A, Attarbaschi A, Niggli F, Zimmermann M, Reiter A, Klapper W (2015) Non-anaplastic peripheral T-cell lymphomas in children and adolescents—a retrospective analysis of the NHL-BFM study group. Br J Haematol 168(6):835–844

    Article  CAS  PubMed  Google Scholar 

  2. Kobayashi R, Yamato K, Tanaka F, Takashima Y, Inada H, Kikuchi A, Kumagai MA, Sunami S, Nakagawa A, Fukano R, Fujita N, Mitsui T, Tsurusawa M (2010) Lymphoma committee, Japanese Pediatric Leukemia/Lymphoma Study Group. Retrospective analysis of non-anaplastic peripheral T-cell lymphoma in pediatric patients in Japan. Pediatr Blood Cancer 54(2):212–215

    PubMed  Google Scholar 

  3. Windsor R, Stilles C, Webb D (2008) Peripheral T-cell lymphomas in childhood: population-based experience in the United Kingdom over 20 years. Pediatr Blood Cancer 50:784–787

    Article  PubMed  Google Scholar 

  4. William BM (2013) Armitage JO international analysis of the frequency and outcome of NK/T-cell lymphomas. Best Pract Res Clin Haematol 26(1):23–32

    Article  CAS  PubMed  Google Scholar 

  5. Intlekofer A, Younes A (2014) From empiric to mechanism-based therapy for peripheral T-cell lymphoma. Int J Hematol 99(3):249–262

    Article  CAS  PubMed  Google Scholar 

  6. Swerdlow SH, Campo E, Harris NL, et al. (2008) WHO classification of Tumours of Haematopoietic and lymphoid tissues. IARC Press, Lyon, France

    Google Scholar 

  7. Attygalle AD, Cabecadas J, Gaulard P, Jaffe E, de Jong D, Ko YH, Said J, Klapper W (2014) Peripheral T-cell and NK-cell lymphomas and their mimics; taking a step forward—report on the lymphoma workshop of the XVIth meeting of the European Association for Haematopathology and the Society for Hematopathology. Histopathology 64(2):171–199

    Article  PubMed  Google Scholar 

  8. Vose J, Armitage J, Weisenburger D (2008) International T-cell lymphoma project. International peripheral T-cell and natural killer/T-cell lymphoma study: pathology findings and clinical outcomes. J Clin Oncol 26(25):4124–4130

    Article  PubMed  Google Scholar 

  9. Hutchinson RE, Laver JH, Chang M, Muzzafar T, Desai S, Murphy S, Schwenn M, Shuster J, Link MP (2008) Non-anaplastic peripheral T-cell lymphoma in childhood and adolescence: a Children’s oncology group study. Pediatr Blood Cancer 51:29–33

    Article  Google Scholar 

  10. Al Mahmoud R, Weitzman S, Schechter T, Ngan B, Abdelhaleem M, Alexander S (2012) Peripheral T-cell lymphomas in children and adolescents: a single-institution experience. J Pediatr Hematol Oncol 34:611–616

    Article  PubMed  Google Scholar 

  11. Sandlund JT, Perkins SL (2015) Uncommon non-Hodgkin lymphomas of childhood: pathological diagnosis, clinical features and treatment approaches. Br J Haematol 169(5):631–646

    Article  PubMed  Google Scholar 

  12. Willemze R (2012) Thirty years of progress in cutaneous lymphoma research. G Ital Dermatol Venereol 147(6):515–521

    CAS  PubMed  Google Scholar 

  13. Oschlies I, Simonitsch-Klupp I, Maldyk J, Konovalov D, Abramov D, Myakova N, Lisfeld J, Attarbaschi A, Kontny U, Woessmann W, Klapper W (2015) Subcutaneous panniculitis-like T-cell lymphoma in children: a detailed clinicopathological description of 11 multifocal cases with a high frequency of haemophagocytic syndrome. Br J Dermatol 172(3):793–797

    Article  CAS  PubMed  Google Scholar 

  14. Sakurai E et al. (2013) Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) with hemophagocytosis (HPS): successful treatment using high-dose chemotherapy (BFM-NHL & ALL-90) and autologous peripheral blood stem cell transplantation. J Clin Exp Hematop 53(2):135–140

    Article  PubMed  Google Scholar 

  15. Willemze R, Dreyling M (2008) Primary cutaneous lymphomas: ESMO clinical practice guidelines for diagnosis, treatment and follow-up. Blood 111:838–845

    Article  CAS  PubMed  Google Scholar 

  16. Belhadj K, Reyes F, Farcet J-P, Tilly H, Bastard C, Angonin R, Deconinck E, Charlotte F, Leblond V, Labouyrie E, Lederlin P, Emile J-F, Delmas-Marsalet B, Arnulf B, Zafrani E-S, Gaulard P (2003) Hepatosplenic γδ T-cell lymphoma is a rare clinicopathological entity with poor outcome: report on a series of 21 patients. Blood 102:4261–4269

    Article  CAS  PubMed  Google Scholar 

  17. Macon WR, Levy NB, Kurtin PJ, Salhany KE, Elkhalifa MY, Casey TT, Craig FE, Vnencak-Jones CL, Gulley ML, Park JP, Cousar JB (2001) Hepatosplenic alphabeta T-cell lymphomas: a report of 14 cases and comparison with hepatosplenic gammadelta T-cell lymphomas. Am J Surg Pathol 25(3):285–296

    Article  CAS  PubMed  Google Scholar 

  18. Wang ZY, Li YX, Wang WH, Jin J, Wang H, Song YW, Liu QF, Wang SL, Liu YP, Qi SN, Fang H, Liu XF, Yu ZH (2009) Primary radiotherapy showed favorable outcome in treating extranodal nasal-type NK/T-cell lymphoma in children and adolescents. Blood 114(23):4771–4776

    Article  CAS  PubMed  Google Scholar 

  19. Ellin F, Landström J, Jerkeman M, Relander T (2014) Real-world data on prognostic factors and treatment in peripheral T-cell lymphomas: a study from the Swedish lymphoma registry. Blood 124(10):1570–1577

    Article  CAS  PubMed  Google Scholar 

  20. Kristinsson SY, Gao Y, Björkholm M, Lund SH, Sjöberg J, Caporaso N, Goldin LR, Landgren O (2015) Hodgkin lymphoma risk following infectious and chronic inflammatory diseases: a large population-based case–control study from Sweden. Int J Hematol 101(6):563–568

    Article  PubMed  Google Scholar 

  21. Biancone L, Onali S, Petruzziello C, Calabrese E, Pallone F (2015) Cancer and immunomodulators in inflammatory bowel diseases. Inflamm Bowel Dis 21(3):674–698

    Article  PubMed  Google Scholar 

Download references

Acknowledgments

We would like to thank all participating institutions and physicians for their support of this study. The EIC-NHL and i-BFM paper was written on behalf of the Berlin-Frankfurt-Münster (BFM) Study Group (Austria, Germany, Switzerland, Czech Republic), Associazione Italiana Ematologica Oncologia Pediatrica (AIEOP), United Kingdom Children’s Cancer and Leukemia Study group (CCLG), Nordic Association of Pediatric Hematology and Oncology (NOPHO) (Norway, Sweden, Denmark, Finland and Iceland), Belgian Society of Pediatric Hematology and Oncology, Dutch Childhood Oncology Group (DCOG), Hungarian Pediatric Oncology Network, Slovakian Pediatric Association (Section of Pediatric Hemato-Oncology), Polish Society of Pediatric Oncology and Hematology, Japanese Pediatric Leukemia/Lymphoma Study Group (JPLSG), Hong Kong Pediatric Hematology and Oncology Group (HKPHOSG), and single institutions from Canada (Toronto), Belarus (Minsk), and Russia (Moscow).

Authorship contributions

DW, LB, AA, UK, and KM designed and planned the study. KM, UK, and AC were in charge of data pooling, data checking, and statistical analysis. All authors were principal or co-investigators in their study groups and institutions, coordinating national trials in their countries, providing study material, and recruiting patients. KM, UK, AA, and UA wrote the manuscript, and all authors read and approved the final version of the manuscript.

Author information

Authors and Affiliations

  1. Department of Pediatric Hematology and Oncology, The Queen Silvia’s Hospital for Children, Rondvägen 10, 41685, Göteborg, Sweden

    K. Mellgren

  2. Pediatric Hematology and Oncology, St. Anna Children’s Hospital, Medical University of Vienna, Vienna, Austria

    A. Attarbaschi

  3. Department of Pediatrics, Division of Hematology and Oncology, Hospital for Sick Children, Toronto, ON, Canada

    O. Abla & S. Alexander

  4. Department of Paediatric Haematology and Oncology, Great North Children’s Hospital, Newcastle Hospitals NHS Foundation Trust, Newcastle upon Tyne, UK

    S. Bomken

  5. Department of Pediatric Oncology and Hematology, University Children’s Hospital, Banska Bystrica, Slovakia

    E. Bubanska

  6. Department of Pediatrics and Adolescent Medicine, Li Ka Shing Faculty of Medicine, Queen Mary Hospital, The University of Hong Kong, Pokfulam, Hong Kong

    A. Chiang

  7. Pediatric Hematology and Oncology, Semmelweis University, Budapest, Hungary

    M. Csóka

  8. Belarusian Research Center for Pediatric Oncology, Hematology and Immunology, Minsk, Belarus

    A. Fedorova

  9. Pediatric Hematology and Oncology, Charles University and University Hospital Motol, Prague, Czech Republic

    E. Kabickova

  10. Clinic of Paediatrics Haemathology and Oncology, Medical University of Gdansk, Gdansk, Poland

    L. Kapuscinska-Kemblowska

  11. Department of Pediatrics, Sapporo Hokuyu Hospital, Sapporo, Japan

    R. Kobayashi

  12. Pediatric Oncology and Hematology, University Hospital, Brno, Czech Republic

    Z. Krenova

  13. Princess Maxima Center for Pediatric Oncology, Utrecht, The Netherlands

    F. Meyer-Wentrup

  14. Pediatric Hematology and Oncology, Federal Center for Pediatric Hematology, Oncology and Immunology, Moscow, Russia

    N. Miakova

  15. Pediatric Hematology and Oncology, University of Padova, Padova, Italy

    M. Pillon

  16. Department of Pediatric Oncology, Hôpital des Enfants, Toulouse, France

    G. Plat

  17. Pediatric Hematology and Oncology, University Hospitals Leuven, Leuven, Belgium

    A. Uyttebroeck

  18. Pediatric Hematology and Oncology, Addbrooke’s Hospital, Cambridge University Hospitals Foundation Trust, Cambridge, UK

    D. Williams

  19. Bone Marrow Transplantation and Pediatric Hematology and Oncology, Wroclaw Medical University, Wroclaw, Poland

    G. Wróbel

  20. Division of Pediatric Hematology, Oncology and Stem Cell Transplantation, University Medical Center, Aachen, Germany

    U. Kontny

Authors
  1. K. Mellgren
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. A. Attarbaschi
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. O. Abla
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. S. Alexander
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. S. Bomken
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. E. Bubanska
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. A. Chiang
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. M. Csóka
    View author publications

    You can also search for this author in PubMed Google Scholar

  9. A. Fedorova
    View author publications

    You can also search for this author in PubMed Google Scholar

  10. E. Kabickova
    View author publications

    You can also search for this author in PubMed Google Scholar

  11. L. Kapuscinska-Kemblowska
    View author publications

    You can also search for this author in PubMed Google Scholar

  12. R. Kobayashi
    View author publications

    You can also search for this author in PubMed Google Scholar

  13. Z. Krenova
    View author publications

    You can also search for this author in PubMed Google Scholar

  14. F. Meyer-Wentrup
    View author publications

    You can also search for this author in PubMed Google Scholar

  15. N. Miakova
    View author publications

    You can also search for this author in PubMed Google Scholar

  16. M. Pillon
    View author publications

    You can also search for this author in PubMed Google Scholar

  17. G. Plat
    View author publications

    You can also search for this author in PubMed Google Scholar

  18. A. Uyttebroeck
    View author publications

    You can also search for this author in PubMed Google Scholar

  19. D. Williams
    View author publications

    You can also search for this author in PubMed Google Scholar

  20. G. Wróbel
    View author publications

    You can also search for this author in PubMed Google Scholar

  21. U. Kontny
    View author publications

    You can also search for this author in PubMed Google Scholar

Consortia

On behalf of the European Intergroup for Childhood Non-Hodgkin Lymphoma (EICNHL) and the international Berlin-Frankfurt-Münster (i-BFM) Group

Corresponding author

Correspondence to K. Mellgren.

Ethics declarations

Conflict of interest

The authors declare that they have no conflict of interest.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Mellgren, K., Attarbaschi, A., Abla, O. et al. Non-anaplastic peripheral T cell lymphoma in children and adolescents—an international review of 143 cases. Ann Hematol 95, 1295–1305 (2016). https://doi.org/10.1007/s00277-016-2722-y

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00277-016-2722-y

Keywords

Search

Navigation