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Pediatric-Type Follicular Lymphoma (PTFL)

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Book cover Non-Hodgkin's Lymphoma in Childhood and Adolescence

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Abstract

Pediatric-type follicular lymphoma (PTFL), as defined in the recently updated 2016 World Health Organization Classification of Tumors of Hematopoietic and Lymphoid Tissues, is a rare entity of non-Hodgkin’s lymphoma (NHL) in childhood and adolescence accounting for less than 2% of all cases. PTFL is a mature B-cell neoplasm that is usually CD20+ and BCL2– and does not carry components of diffuse large B-cell lymphoma or rearrangements of BCL2, BCL6, IRF4, and IG loci. The most important histopathological differential diagnosis is large B-cell lymphoma with IRF4 rearrangement, which may be clinically indistinguishable from PTFL. PTFL is associated with male gender, adolescent age, normal serum LDH levels, lack of B-symptoms and limited stage I or II disease, mostly involving peripheral lymph nodes in the head and neck region. Treatment outcomes in PTFL seem to be excellent with either complete resection and an observational strategy only, or with risk-adapted B-cell NHL-type chemotherapy resulting in overall survival rates >95%.

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Authors and Affiliations

  1. Department of Pediatric Hematology and Oncology, St. Anna Children’s Hospital, Vienna, Austria

    Andishe Attarbaschi

  2. Department of Pediatrics and Adolescent Medicine, Medical University of Vienna, Vienna, Austria

    Andishe Attarbaschi

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  1. Andishe Attarbaschi
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Correspondence to Andishe Attarbaschi .

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Editors and Affiliations

  1. University of Toronto, The Hospital for Sick Children, Toronto, ON, Canada

    Oussama Abla

  2. Medical University of Vienna, St. Anna Children’s Hospital, Vienna, Wein, Austria

    Andishe Attarbaschi

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Attarbaschi, A. (2019). Pediatric-Type Follicular Lymphoma (PTFL). In: Abla, O., Attarbaschi, A. (eds) Non-Hodgkin's Lymphoma in Childhood and Adolescence. Springer, Cham. https://doi.org/10.1007/978-3-030-11769-6_16

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  • DOI: https://doi.org/10.1007/978-3-030-11769-6_16

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  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-11768-9

  • Online ISBN: 978-3-030-11769-6

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