Skip to main content
SpringerLink
Log in
Book cover

Atlas of Bone Marrow Pathology pp 129–157Cite as

Lymphoblastic Leukemia/Lymphoma

  • Chapter
  • First Online:

  • 2451 Accesses

Part of the book series: Atlas of Anatomic Pathology ((AAP))

Abstract

Lymphoblastic leukemia/lymphoma (i.e., acute lymphoblastic leukemia/lymphoma or ALL) is the most common type of cancer in childhood. The age-adjusted incidence rate is 3.1/100,000 in children, so approximately 2500–3000 children are diagnosed with acute lymphoblastic leukemia (ALL) each year in the United States). Approximately 80–85% of the cases are B-ALL and 10–15% are T-ALL (Table 9.1). Approximately 75% of ALL occurs in children under 6 years old, but it can occur at any age [1, 2]. Patients typically present with anemia, thrombocytopenia, and/or neutropenia-related signs and symptoms such as fatigue, weakness, shortness of breath, easy bruising, bleeding, and infection. Genetic factors play important roles in cancer initiation. Age, WBC count at disease presentation and genetic aberrations dictate risk stratification.

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

Chapter
USD   29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD   109.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD   139.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD   219.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Learn about institutional subscriptions

References

  1. Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, et al. WHO classification of tumours of haematopoietic and lymphoid tissues. 4th ed. IARC: Lyon; 2008. p. 168–78.

    Google Scholar 

  2. Bassan R, Maino E, Cortelazzo S. Lymphoblastic lymphoma: an updated review on biology, diagnosis, and treatment. Eur J Haematol. 2016;96:447–60. https://doi.org/10.1111/ejh.12722.

    Article  PubMed  Google Scholar 

  3. Moriyama T. Familial acute lymphoblastic leukemia. Rinsho Ketsueki. 2016;57:900–9. 10.11406/rinketsu.57.900.

    PubMed  Google Scholar 

  4. Pulte D, Gondos A, Brenner H. Improvement in survival in younger patients with acute lymphoblastic leukemia from the 1980s to the early 21st century. Blood. 2009;113:1408–11. https://doi.org/10.1182/blood-2008-06-164863.

    Article  CAS  PubMed  Google Scholar 

  5. Pui CH, Mullighan CG, Evans WE, Relling MV. Pediatric acute lymphoblastic leukemia: where are we going and how do we get there? Blood. 2012;120:1165–74. https://doi.org/10.1182/blood-2012-05-378943.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  6. Madhusoodhan PP, Carroll WL, Bhatla T. Progress and prospects in pediatric leukemia. Curr Probl Pediatr Adolesc Health Care. 2016;46:229–41. https://doi.org/10.1016/j.cppeds.2016.04.003.

    Article  PubMed  Google Scholar 

  7. Sorensen JT, Gerald K, Bodensteiner D, Holmes FF. Effect of age on survival in acute leukemia. 1950-1990. Cancer. 1993;72:1602–6.

    Article  CAS  PubMed  Google Scholar 

  8. Irken G, Oren H, Gülen H, Duman M, Uçar C, Atabay B, et al. Treatment outcome of adolescents with acute lymphoblastic leukemia. Ann Hematol. 2002;81:641–5.

    Article  CAS  PubMed  Google Scholar 

  9. Jacobson S, Tedder M, Eggert J. Adult acute lymphoblastic leukemia: a genetic overview and application to clinical practice. Clin J Oncol Nurs. 2016;20:E147–54.

    Article  PubMed  Google Scholar 

  10. Paul S, Kantarjian H, Jabbour EJ. Adult acute lymphoblastic leukemia. Mayo Clin Proc. 2016;91:1645–66. https://doi.org/10.1016/j.mayocp.2016.09.010.

    Article  PubMed  Google Scholar 

  11. Guest EM, Stam RW. Updates in the biology and therapy for infant acute lymphoblastic leukemia. Curr Opin Pediatr. 2017;29:20–6. https://doi.org/10.1097/MOP.0000000000000437.

    Article  CAS  PubMed  Google Scholar 

  12. Safavi S, Paulsson K. Near-haploid and low-hypodiploid acute lymphoblastic leukemia: two distinct subtypes with consistently poor prognosis. Blood. 2017;129:420–3. https://doi.org/10.1182/blood-2016-10-743765.

    Article  CAS  PubMed  Google Scholar 

  13. Bhojwani D, Kang H, Menezes RX, Yang W, Sather H, Moskowitz NP, et al.; Children’s Oncology Group Study; Dutch Childhood Oncology Group; German Cooperative Study Group for Childhood Acute Lymphoblastic Leukemia. Gene expression signatures predictive of early response and outcome in high-risk childhood acute lymphoblastic leukemia: a Children’s Oncology Group Study [corrected]. J Clin Oncol 2008;26:4376–4384. doi: https://doi.org/10.1200/JCO.2007.14.4519

  14. Moorman AV, Schwab C, Ensor HM, Russell LJ, Morrison H, Jones L, et al. IGH@ translocations, CRLF2 deregulation, and microdeletions in adolescents and adults with acute lymphoblastic leukemia. J Clin Oncol. 2012;30:3100–8. https://doi.org/10.1200/JCO.2011.40.3907.

    Article  PubMed  Google Scholar 

  15. Roberts KG, Morin RD, Zhang J, Hirst M, Zhao Y, Su X, et al. Genetic alterations activating kinase and cytokine receptor signaling in high-risk acute lymphoblastic leukemia. Cancer Cell. 2012;22:153–66. https://doi.org/10.1016/j.ccr.2012.06.005.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  16. Mullighan CG. The genomic landscape of acute lymphoblastic leukemia in children and young adults. Hematology Am Soc Hematol Educ Program. 2014;2014(1):174–80. https://doi.org/10.1182/asheducation-2014.1.174.

    PubMed  Google Scholar 

  17. Roberts KG, Li Y, Payne-Turner D, Harvey RC, Yang YL, Pei D, et al. Targetable kinase-activating lesions in Ph-like acute lymphoblastic leukemia. N Engl J Med. 2014;371:1005–15. https://doi.org/10.1056/NEJMoa1403088.

    Article  PubMed  PubMed Central  Google Scholar 

  18. Boer JM, Steeghs EM, Marchante JR, Boeree A, Beaudoin JJ, Beverloo HB, et al. Tyrosine kinase fusion genes in pediatric BCR-ABL1-like acute lymphoblastic leukemia. Oncotarget. 2017;8:4618–28. 10.18632/oncotarget.13492.

    Article  PubMed  Google Scholar 

  19. Den Boer ML, van Slegtenhorst M, De Menezes RX, Cheok MH, Buijs-Gladdines JG, Peters ST, et al. A subtype of childhood acute lymphoblastic leukaemia with poor treatment outcome: a genome-wide classification study. Lancet Oncol. 2009;10:125–34. https://doi.org/10.1016/S1470-2045(08)70339-5.

    Article  Google Scholar 

  20. Jain N, Roberts KG, Jabbour E, Patel K, Eterovic AK, Chen K, et al. Ph-like acute lymphoblastic leukemia: a high-risk subtype in adults. Blood. 2017;129:572–81. https://doi.org/10.1182/blood-2016-07-726588.

    Article  CAS  PubMed  Google Scholar 

  21. Roberts KG, Gu Z, Payne-Turner D, McCastlain K, Harvey RC, Chen IM, et al. High frequency and poor outcome of Philadelphia chromosome–like acute lymphoblastic leukemia in adults. J Clin Oncol. 2017;35:394–401. https://doi.org/10.1200/JCO.2016.69.0073.

    Article  PubMed  Google Scholar 

  22. Ryan SL, Matheson E, Grossmann V, Sinclair P, Bashton M, Schwab C, et al. The role of the RAS pathway in iAMP21-ALL. Leukemia. 2016;30:1824–31. https://doi.org/10.1038/leu.2016.80.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  23. Buitenkamp TD, Izraeli S, Zimmermann M, Forestier E, Heerema NA. van den Heuvel-Eibrink MM, et al. Acute lymphoblastic leukemia in children with Down syndrome: a retrospective analysis from the Ponte di Legno study group. Blood. 2014;123:70–7. https://doi.org/10.1182/blood-2013-06-509463.

  24. Lee P, Bhansali R, Izraeli S, Hijiya N, Crispino JD. The biology, pathogenesis and clinical aspects of acute lymphoblastic leukemia in children with Down syndrome. Leukemia. 2016;30:1816–23. https://doi.org/10.1038/leu.2016.164.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  25. Zhang L, Ramjit RT, Hill CE, Arellano M, Khoury HJ, Mann KP. Clinical significance of quantitative monitoring and mutational analysis of BCR-ABL1 transcript in Philadelphia chromosome positive B lymphoblastic leukemia. Leuk Lymphoma. 2015;57:364–9. https://doi.org/10.3109/10428194.2014.1003059.

    Article  Google Scholar 

  26. Short NJ, Jabbour E, Sasaki K, Patel K, O’Brien SM, Cortes JE, et al. Impact of complete molecular response on survival in patients with Philadelphia chromosome–positive acute lymphoblastic leukemia. Blood. 2016;128:504–7. https://doi.org/10.1182/blood-2016-03-707562.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  27. Belver L, Ferrando A. The genetics and mechanisms of T cell acute lymphoblastic leukaemia. Nat Rev Cancer. 2016;16:494–507. https://doi.org/10.1038/nrc.2016.63.

    Article  CAS  PubMed  Google Scholar 

  28. Karrman K, Johansson B. Pediatric T-cell acute lymphoblastic leukemia. Genes Chromosomes Cancer. 2017;56:89–116. https://doi.org/10.1002/gcc.22416.

    Article  CAS  PubMed  Google Scholar 

  29. Heerema NA, Sather HN, Sensel MG, Kraft P, Nachman JB, Steinherz PG, et al. Frequency and clinical significance of cytogenetic abnormalities in pediatric T-lineage acute lymphoblastic leukemia: a report from the Children’s Cancer Group. J Clin Oncol. 1998;16:1270–8.

    Article  CAS  PubMed  Google Scholar 

  30. Schneider NR, Carroll AJ, Shuster JJ, Pullen DJ, Link MP, Borowitz MJ, et al. New recurring cytogenetic abnormalities and association of blast cell karyotypes with prognosis in childhood T-cell acute lymphoblastic leukemia: a pediatric oncology group report of 343 cases. Blood. 2000;96:2543–9.

    CAS  PubMed  Google Scholar 

  31. Karrman K, Forestier E, Heyman M, Andersen MK, Autio K, Blennow E, et al. Clinical and cytogenetic features of a population-based consecutive series of 285 pediatric T-cell acute lymphoblastic leukemias: rare T-cell receptor gene rearrangements are associated with poor outcome. Genes Chromosomes Cancer. 2009;48:795–805. https://doi.org/10.1002/gcc.20684.

    Article  CAS  PubMed  Google Scholar 

  32. Girardi T, Vicente C, Cools J, De Keersmaecker K. The genetics and molecular biology of T-ALL. Blood. 2017;129:1113–23. https://doi.org/10.1182/blood-2016-10-706465.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  33. Reichard K. Precursor B- and T-cell acute lymphoblastic leukemia/lymphoma (aka lymphoblastic leukemia/lymphoma). In: Foucar K, Reichard K, Czuchlewski D, editors. Bone marrow pathology. 3rd ed. Chicago: ASCP; 2010. p. 591–615.

    Google Scholar 

  34. Suzuki S, Nagel S, Schneider B, Chen S, Kaufmann M, Uozumi K, et al. A second NOTCH1 chromosome rearrangement: t(9;14)(q34.3;q11.2) in T-cell neoplasia. Leukemia. 2009;23:1003–6. https://doi.org/10.1038/leu.2008.366.

    Article  CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Pathology, University of New Mexico, Albuquerque, NM, USA

    Qian-Yun Zhang

Authors
  1. Qian-Yun Zhang
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Qian-Yun Zhang .

Editor information

Editors and Affiliations

  1. Department of Pathology, University of New Mexico School of Medicine, Albuquerque, New Mexico, USA

    Tracy I. George

  2. Department of Pathology, The University of Chicago, Chicago, Illinois, USA

    Daniel A. Arber

Rights and permissions

Reprints and permissions

Copyright information

© 2018 Springer Science+Business Media, LLC

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Zhang, QY. (2018). Lymphoblastic Leukemia/Lymphoma. In: George, T., Arber, D. (eds) Atlas of Bone Marrow Pathology. Atlas of Anatomic Pathology. Springer, New York, NY. https://doi.org/10.1007/978-1-4939-7469-6_9

Download citation

  • DOI: https://doi.org/10.1007/978-1-4939-7469-6_9

  • Published:

  • Publisher Name: Springer, New York, NY

  • Print ISBN: 978-1-4939-7467-2

  • Online ISBN: 978-1-4939-7469-6

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation