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    Childhood Non-Hodgkin Lymphoma Treatment (PDQ®): Treatment - Health Professional Information [NCI] - Cellular Classification of Childhood NHL

    Cellular Classification and Clinical Presentation

    In children, non-Hodgkin lymphoma (NHL) is distinct from the more common forms of lymphoma observed in adults. While lymphomas in adults are more commonly low or intermediate grade, almost all NHL that occurs in children is high grade.[1,2,3] The World Health Organization (WHO) has classified NHL on the basis of the following: (1) phenotype (i.e., B-lineage and T-lineage or natural killer [NK] cell lineage) and (2) differentiation (i.e., precursor vs. mature).[4]

    On the basis of clinical response to treatment, NHL of childhood and adolescence currently falls into the following three therapeutically relevant categories:

    1. Mature B-cell NHL (Burkitt and Burkitt-like lymphoma/leukemia and diffuse large B-cell lymphoma).
    2. Lymphoblastic lymphoma (primarily precursor T-cell lymphoma and, less frequently, precursor B-cell lymphoma).
    3. Anaplastic large cell lymphoma (mature T-cell or null-cell lymphomas).

    NHL associated with immunodeficiency generally has a mature B-cell phenotype and is more often of large cell than Burkitt histology.[5] Posttransplant lymphoproliferative diseases are classified according to WHO nomenclature as (1) early lesions, (2) polymorphic, and (3) monomorphic.[6] While the majority of posttransplant lymphoproliferative diseases are of B-cell phenotype, approximately 10% are mature (peripheral) T-cell lymphomas.[6]

    Other types of lymphoma, such as peripheral T-cell lymphoma, T/NK lymphomas, cutaneous lymphomas, and indolent B-cell lymphomas (e.g., follicular lymphoma), are more commonly seen in adults and occur rarely in children. Refer to the following PDQ summaries for more information:

    • Adult Non-Hodgkin Lymphoma.
    • Primary CNS Lymphoma.
    • Mycosis Fungoides and the Sézary Syndrome.

    Each type of childhood NHL is associated with distinctive molecular biological characteristics, which are outlined in the following table. The Revised European-American Lymphoma (REAL) classification and the WHO classification are the most current NHL classifications utilized and are shown below.[2] The Working Formulation is also listed for reference. The WHO classification applies the principles of the REAL classification and focuses on the specific type of lymphoma for therapy purposes. For the most part, the remaining categories do not pertain to pediatric NHL and are not shown.

    Table 2. Major Histopathological Categories of Non-Hodgkin Lymphoma in Children and Adolescentsa

    Category (WHO Classification/ Updated REAL) Category (Working Formulation) Immuno-phenotype Clinical Presentation Chromosome Translocation Genes Affected
    CNS = central nervous system; ML = malignant lymphoma; REAL = Revised European-American Lymphoma; WHO = World Health Organization.
    a Adapted from Percy et al.[2]
    Burkitt and Burkitt-like lymphomas ML small noncleaved cell Mature B cell Intra-abdominal (sporadic), head and neck (non-jaw, sporadic), jaw (endemic), bone marrow, CNS t(8;14)(q24;q32), t(2;8)(p11;q24), t(8;22)(q24;q11) C-MYC,IGH,IGK,IGL
    Diffuse large B-cell lymphoma ML large cell Mature B cell; maybe CD30+ Nodal, abdominal, bone, primary CNS (when associated with immunodeficiency), mediastinal No consistent cytogenetic abnormality identified
    Lymphoblastic lymphoma, precursor T-cell leukemia, or precursor B-cell lymphoma Lymphoblastic convoluted and non-convoluted Pre-T cell Mediastinal, bone marrow MTS1/p16ink4a; DeletionTAL1t(1;14)(p34;q11), t(11;14)(p13;q11) TAL1, TCRAO, RHOMB1, HOX11
    Pre-B cell Skin, bone, mediastinal
    Anaplastic large cell lymphoma, systemic ML immunoblastic or ML large CD30+ (Ki-1+) Variable, but systemic symptoms often prominent t(2;5)(p23;q35); less common variant translocations involvingALK ALK,NPM
    T cell or null cell
    Anaplastic large cell lymphoma, cutaneous CD30+ (Ki-usually) Skin only; single or multiple lesions Lacks t(2;5)
    T cell
    1 | 2 | 3 | 4 | 5 | 6 | 7 | 8 | 9
    1 | 2 | 3 | 4 | 5 | 6 | 7 | 8 | 9
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