title: Primary Effusion Lymphoma (PEL)-Like Lymphoma in a Child With Congenital Immunodeficiency.
abstract: Primary effusion lymphoma (PEL) is a rare lymphoma that occurs more frequently in immunocompromised adults and has a poor survival. We report a 9-year-old female with combined immunodeficiency with an Epstein-Barr virus positive/human herpes virus 8 negative PEL-like lymphoma. The treatment with systemic chemotherapy for non-Hodgkin lymphoma, zidovudine, and interferon-α failed to control disease progression. This is the first reported pediatric case of PEL-like lymphoma. Increased diagnostic awareness and more effective treatment strategies are needed for this rare lymphoma.
authors.affiliation: Division of Haematology/Oncology, The Hospital for Sick Children, Toronto, Ontario, Canada.
authors.forename: Grace K S
authors.initials: GK
authors.lastname: Lam
authors.affiliation: Department of Paediatric Laboratory Medicine, The Hospital for Sick Children, Toronto, Ontario, Canada.
authors.forename: Mohamed
authors.initials: M
authors.lastname: Abdelhaleem
authors.affiliation: Department of Paediatric Laboratory Medicine, The Hospital for Sick Children, Toronto, Ontario, Canada.
authors.forename: Gino R
authors.initials: GR
authors.lastname: Somers
authors.affiliation: Division of Immunology and Allergy, The Hospital for Sick Children, Toronto, Ontario, Canada.
authors.forename: Chaim
authors.initials: C
authors.lastname: Roifman
authors.affiliation: Division of Infectious Diseases, The Hospital for Sick Children, Toronto, Ontario, Canada.
authors.forename: Stanley
authors.initials: S
authors.lastname: Read
authors.affiliation: Division of Haematology/Oncology, The Hospital for Sick Children, Toronto, Ontario, Canada.
authors.forename: Oussama
authors.initials: O
authors.lastname: Abla
mesh_terms: D002648:Child; D005260:Female; D006801:Humans; D007153:Immunologic Deficiency Syndromes / Q000150:complications* / Q000151:congenital; D054685:Lymphoma, Primary Effusion / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology*
keywords: EBV; children; lymphoma; primary effusion