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American Academy of Pediatrics
Article

Pediatric Lymphoma

Ilia N. Buhtoiarov
Pediatrics in Review September 2017, 38 (9) 410-423; DOI: https://doi.org/10.1542/pir.2016-0152
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Ilia N. Buhtoiarov
*Pediatric Leukemia and Lymphoma Clinic, Cleveland Clinic Children’s Hospital, Cleveland, OH
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  1. Ilia N. Buhtoiarov, MD*
  1. *Pediatric Leukemia and Lymphoma Clinic, Cleveland Clinic Children’s Hospital, Cleveland, OH
  • AUTHOR DISCLOSURE

    Dr Buhtoiarov has disclosed no financial relationships relevant to this article. This commentary does not contain a discussion of an unapproved/investigative use of a commercial product/device.

  • Abbreviations:
    CAR:
    chimeric antigen receptor
    cHL:
    classic Hodgkin lymphoma
    CNS:
    central nervous system
    CT:
    computed tomography
    EBV:
    Epstein-Barr virus
    HIV:
    human immunodeficiency virus
    HL:
    Hodgkin lymphoma
    HRSC:
    Hodgkin Reed-Sternberg cell
    HSCT:
    hematopoietic stem cell transplantation
    Ig:
    immunoglobulin
    LN:
    lymph node
    NHL:
    non-Hodgkin lymphoma
    NLPD:
    nodular lymphocyte–predominant
    SVC:
    superior vena cava
    TLS:
    tumor lysis syndrome
    UA:
    uric acid
  • Education Gaps

    Painless lymphadenopathy is one of the commonest presentations of pediatric lymphoma. Absence of the absolute lymphoma-specific signs and symptoms makes it a particular diagnostic challenge. Lack of systemic symptoms does not preclude a malignant transformation. High level of suspicion is critical for timely patient referral to a pediatric oncologist. Outstanding survival rates may be compromised by a substantial prevalence of the therapy-related side effects.

    Objectives

    After completing the article, readers should be able to:

    1. Recognize genetic and environmental factors contributing to development of lymphoma.

    2. Identify clinical parameters that can be used to predict the nonbenign nature of lymphadenopathy.

    3. Recognize lymphoma-associated oncologic emergencies.

    4. Diagnose tumor lysis syndrome and propose prophylactic and therapeutic interventions.

    5. Discuss therapeutic options and recognize therapy-related side effects.

    Introduction

    Lymphoma is the third most frequent childhood malignancy (prevalence rate of 12%–15%), closely following acute leukemia and central nervous system (CNS) tumors. Most pediatric patients with lymphoma will survive their disease into adulthood. Having a high threshold of clinical suspicion at the time of first assessment, along with performing problem-oriented initial tests, followed by prompt referral to the pediatric lymphoma expert for further evaluation and specialized treatment, are the pillars of therapeutic success. This review will serve to update the readership on pediatric lymphoma epidemiology and known predisposition factors, clinical presentation, diagnostic tests, and therapeutic options, as well as treatment-related side effects that may need to be recognized while taking care of lymphoma survivors.

    Lymphoma is a neoplasm caused by malignant transformation of lymphoid cells. Advances in the understanding of lymphoma biology led to development of risk- and response-adapted therapies, which caused lymphomas to be one of the most curable pediatric cancers. The …

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    In this issue

    Pediatrics in Review: 38 (9)
    Pediatrics in Review
    Vol. 38, Issue 9
    1 Sep 2017
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    Pediatric Lymphoma
    Ilia N. Buhtoiarov
    Pediatrics in Review Sep 2017, 38 (9) 410-423; DOI: 10.1542/pir.2016-0152

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    Pediatric Lymphoma
    Ilia N. Buhtoiarov
    Pediatrics in Review Sep 2017, 38 (9) 410-423; DOI: 10.1542/pir.2016-0152
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    • Article
      • Education Gaps
      • Objectives
      • Introduction
      • Biology and Epidemiology of Lymphoma
      • Clinical Presentation
      • Oncologic Emergencies in Newly Diagnosed Lymphoma
      • Approaches to Diagnosis
      • Lymphoma Therapy: Risk- and Response-Adapted Therapeutic Approaches
      • Disease Surveillance After Therapy Completion
      • Late Effects of Therapy
      • Conclusions and Further Directions
      • References
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