title: Subsequent development of diffuse large B-cell lymphomas and Hodgkin lymphoma associated with primary immune disorder in a 6-year-old female: a case report and review of the literature.
abstract: Neoplastic lymphoid proliferation may arise from immune deficiency or disordered regulation of the immune system. Often the neoplasms are associated with viral agents, such as Epstein-Barr virus, human immunodeficiency virus, or human herpes virus 8. Lymphoproliferative diseases have been documented in a variety of primary immune disorders. The most commonly encountered neoplastic lesion is diffuse large B-cell lymphoma (DLBCL), although Hodgkin lymphoma (HL), Burkitt lymphoma, and peripheral T-cell lymphomas and/or leukemias have also been documented in rare instances. We report a case of a 6-year-old girl with unclassifiable primary immunodeficiency diagnosed with 2 different clones of DLBCLs and subsequently developed lymphocyte-depleted, classical HL. Both neoplasms were associated with Epstein-Barr virus. To the best of our knowledge, this is the first reported occurrence of primary immune disorder-associated lymphoproliferative disease with sequential development of DLBCLs and HL in a pediatric patient. Thorough surveillance is paramount for accurate assessment of the associated lymphoproliferative disease and in ascertaining likely transformation to, or de novo evolution of a different lymphoid neoplasm. This is also important in evaluating treatment response with appropriate therapeutic adjustments if clinically indicated.
authors.affiliation: Departments of Pathology and Laboratory Medicine, Loma Linda University Medical Center, Loma Linda, CA 92354, USA.
authors.forename: Marnelli A
authors.initials: MA
authors.lastname: Bautista-Quach
authors.forename: Antranik
authors.initials: A
authors.lastname: Bedros
authors.forename: Jun
authors.initials: J
authors.lastname: Wang
mesh_terms: D002648:Child; D005260:Female; D006689:Hodgkin Disease / Q000150:complications* / Q000175:diagnosis / Q000821:virology; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000150:complications* / Q000175:diagnosis / Q000821:virology; D016403:Lymphoma, Large B-Cell, Diffuse / Q000150:complications* / Q000175:diagnosis / Q000821:virology