title: Facial manifestations of Epstein-Barr virus-related lymphoproliferative disease in childhood acute lymphoblastic leukemia in remission: Two atypical presentations.
abstract: Epstein-Barr virus-related lymphoproliferative disease (EBV-LPD) rarely occurs in patients with acute lymphoblastic leukemia (ALL), who have not received hematopoietic transplantation. We describe EBV-LPD manifesting as facial lesions in two children with ALL in remission. One patient was a 16-year-old male with T-cell ALL with an EBV-positive angiocentric polymorphous lip lesion presenting as right-sided facial swelling. The other patient was a 12-year-old male with B-cell ALL with an EBV-positive polymorphous lymphoplasmacytic infiltrate presenting as bilateral dacryoadenitis. Neither patient had known primary immunodeficiencies. Both cases improved with immunosuppressant de-escalation. These cases suggest that immunosuppression induced by maintenance chemotherapy is sufficient to promote EBV-LPD.
authors.affiliation: Division of Pediatric Hematology & Oncology, Massachusetts General Hospital for Children, Harvard Medical School, Boston, Massachusetts.
authors.forename: Benjamin Y
authors.initials: BY
authors.lastname: Lu
authors.affiliation: Center for Transplantation Sciences, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts.
authors.forename: Lisa
authors.initials: L
authors.lastname: Kojima
authors.affiliation: Division of Pediatric Hematology & Oncology, Massachusetts General Hospital for Children, Harvard Medical School, Boston, Massachusetts.
authors.forename: Mary S
authors.initials: MS
authors.lastname: Huang
authors.affiliation: Division of Pediatric Hematology & Oncology, Massachusetts General Hospital for Children, Harvard Medical School, Boston, Massachusetts.
authors.forename: Alison M
authors.initials: AM
authors.lastname: Friedmann
authors.affiliation: Department of Pathology, Massachusetts General Hospital, Harvard Medical School, Boston, Massachusetts.
authors.forename: Judith A
authors.initials: JA
authors.lastname: Ferry
authors.affiliation: Division of Pediatric Hematology & Oncology, Massachusetts General Hospital for Children, Harvard Medical School, Boston, Massachusetts. hweinstein@mgh.harvard.edu.
authors.forename: Howard J
authors.initials: HJ
authors.lastname: Weinstein
mesh_terms: D000293:Adolescent; D002648:Child; D003607:Dacryocystitis / Q000209:etiology*; D020031:Epstein-Barr Virus Infections / Q000150:complications*; D006801:Humans; D008232:Lymphoproliferative Disorders / Q000150:complications*; D008297:Male; D054198:Precursor Cell Lymphoblastic Leukemia-Lymphoma / Q000150:complications*
keywords: Epstein-Barr virus; acute lymphoblastic leukemia; lymphoproliferative disease; maintenance chemotherapy